Materiały źródłowe

• #1 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Adult-onset Stills disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. […] The first part of this review deals with the treatments that are currently available for AOSD. […] Given that the current information on treatment efficacy is obtained from small retrospective case series and not from prospective, double-blind, randomized trials, the treatment of AOSD remains empirical. […] Corticosteroids remain the first-line treatment for AOSD, regardless of the clinical presentation. […] In the event of failure of corticosteroid treatment or steroid-dependence, disease-modifying anti-rheumatic drugs (DMARDS) can be considered. […] Methotrexate has proved beneficial and remains the first-line steroid-sparing treatment in AOSD.

• #2 Adult-Onset Still’s Disease: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/adult-onset-stills-disease-aosd

  Adult-onset Stills disease is a rare type of inflammatory arthritis that develops unexpectedly in early adulthood. Anti-inflammatory medications can help suppress the inflammation. […] Healthcare providers treat adult-onset Stills disease with various types of anti-inflammatory drugs. Different medications work better for different people. It can take some trial and error to find which ones work best to manage your symptoms. Once you do, you may need to continue taking them for a long time, even when you’re not having symptoms. This helps to keep the inflammation from building up again. […] Medications can’t cure AOSD, but they can help suppress your symptoms. For some people, symptoms will eventually go away and not come back. This is called remission. If you go into remission, you can discontinue your medications. For some people, AOSD never really goes away. You may need to take medications indefinitely to keep the inflammation under control.

• #3 Adult-Onset Still’s Disease—A Complex Disease, a Challenging Treatment

  https://www.mdpi.com/1422-0067/23/21/12810

  Adult-onset Still’s disease (AOSD) is a systemic inflammatory disorder with an unknown cause characterized by high-spiking fever, lymphadenopathy, hepatosplenomegaly, hyperferritinemia, and leukocytosis. […] There are no treatment guidelines for AOSD due to its rarity, absence of controlled research, and lack of a standard definition for remission and therapy objectives. Non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids (CS), and conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) are used in AOSD treatment. Biological therapy, including IL-1, IL-6, IL-18, and IL-17 inhibitors, as well as TNFα or Janus-kinases (JAKs) inhibitors, is administered to patients who do not react to CS and csDMARDs or achieve an inadequate response. […] The primary objectives of treatment for AOSD are to reduce inflammation and promote the resolution of systemic and articular symptoms and prevent organ damage and MAS.

• #4 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  Adult-onset Stills disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis; most doctors treating AOSD in the Netherlands treat 5 patients per year. Currently, there is no internationally accepted treatment guideline for AOSD. […] The panel consented on reduction of the use of glucocorticoids to avoid side effects, and preferred the use of biologics over conventional treatment. The role of IL-1 and IL-6 blocking agents was considered important in the treatment of AOSD. […] Anti-inflammatory drugs form the cornerstone of treatment of AOSD. Treatment is often based on expert opinion through the lack of international guidelines. Historically, glucocorticoids (GC) are considered the backbone of treatment. MTX is often the first conventional DMARD used to spare GC. More recently, IL-1 and IL-6 were identified as important mediators of inflammation in AOSD. Biologics targeting these pathways are approved by both the FDA and the EMA for the treatment of sJIA and AOSD.

• #5 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Adult-onset Stills disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. […] The first part of this review deals with the treatments that are currently available for AOSD. […] Given that the current information on treatment efficacy is obtained from small retrospective case series and not from prospective, double-blind, randomized trials, the treatment of AOSD remains empirical. […] Corticosteroids remain the first-line treatment for AOSD, regardless of the clinical presentation. […] In the event of failure of corticosteroid treatment or steroid-dependence, disease-modifying anti-rheumatic drugs (DMARDS) can be considered. […] Methotrexate has proved beneficial and remains the first-line steroid-sparing treatment in AOSD.

• #6 Adult-onset Still’s disease: Treatment – UpToDate

  https://www.uptodate.com/contents/treatment-of-adult-onset-stills-disease

  Adult-onset Still’s disease: Treatment […] The treatment and prognosis of AOSD will be reviewed here. […] The goals of therapy include: […] Control of the physical signs and symptoms of inflammation (eg, fever, rash, morning stiffness, joint pain, and swelling) and, secondarily, control of laboratory indices of inflammation (eg, elevations in the erythrocyte sedimentation rate [ESR] and levels of C-reactive protein [CRP]).

• #7 Adult-onset Still’s disease | Symptoms, treatments and new research

  https://versusarthritis.org/about-arthritis/conditions/adult-onset-still-s-disease/

  Once you have been diagnosed, you will be seen at a rheumatology clinic in a hospital. Youll usually see a specialist doctor, known as a rheumatologist, and a rheumatology nurse specialist. […] Your rheumatology team will be able to advise you on treatments and things you can do for yourself to reduce the symptoms of AOSD. […] The main drugs used to treat AOSD are: non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying anti-rheumatic drugs (DMARDs), steroids, biological therapies. […] NSAIDs will be the first treatment for people who have a mild form of AOSD. Theyre effective at reducing the symptoms of fever and arthritis, but most people will need to take NSAIDs alongside other drugs to bring the condition fully under control. […] Steroids will often be given as one of the first treatments for people with AOSD, particularly if the symptoms are severe, as they help to reduce symptoms quickly in many people with the condition.

• #8 Successful Treatment of Recurrent Adult-Onset Still’s Disease wi | CCID

  https://www.dovepress.com/successful-treatment-of-recurrent-adult-onset-stills-disease-with-toci-peer-reviewed-fulltext-article-CCID

  Adult-onset Stills disease (AOSD) is considered a rare autoimmune inflammatory disorder with an unclear etiology and pathogenesis. […] In terms of treatment, glucocorticoids have always been the cornerstone medication, but some patients exhibit suboptimal responses to conventional drug therapy, making disease control challenging. […] However, as our understanding of the pathogenesis continues to grow, novel therapeutic approaches targeting various cytokines have been gradually identified. […] In this report, we present a case of successful treatment of recurrent AOSD with tocilizumab (TCZ), along with a concise review of innovative treatment strategies for AOSD based on literature retrieval. […] Glucocorticoids are the mainstay of treatment for AOSD. However, as understanding of the disease deepens, novel therapeutic modalities, such as tumor necrosis factor-alpha (TNF-) inhibitors and interleukin inhibitors, have emerged.

• #9 Adult Still disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/diagnosis-treatment/drc-20351912

  A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects. […] Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function. […] Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes. […] The medicine methotrexate (Trexall) is often used in combination with prednisone. The prednisone dose is reduced when combined with methotrexate.

• #10 Adult-onset Still’s disease | Symptoms, treatments and new research

  https://versusarthritis.org/about-arthritis/conditions/adult-onset-still-s-disease/

  Once you have been diagnosed, you will be seen at a rheumatology clinic in a hospital. Youll usually see a specialist doctor, known as a rheumatologist, and a rheumatology nurse specialist. […] Your rheumatology team will be able to advise you on treatments and things you can do for yourself to reduce the symptoms of AOSD. […] The main drugs used to treat AOSD are: non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying anti-rheumatic drugs (DMARDs), steroids, biological therapies. […] NSAIDs will be the first treatment for people who have a mild form of AOSD. Theyre effective at reducing the symptoms of fever and arthritis, but most people will need to take NSAIDs alongside other drugs to bring the condition fully under control. […] Steroids will often be given as one of the first treatments for people with AOSD, particularly if the symptoms are severe, as they help to reduce symptoms quickly in many people with the condition.

• #11 Adult Still disease | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/adult-still-disease

  Adult Still disease can damage joints, particularly the wrists. Treatment involves medicine to reduce pain and help control the disease. Prednisone is often used if pain relievers such as ibuprofen (Advil, Motrin IB, others) are not enough. […] A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects. […] Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function. […] Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes.

• #12 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Testing prior to treatment initiation: Prior to initiation of any therapy, a complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) levels, serum creatinine levels, and liver profile should be ordered. The treatment options provided below are listed in the order of management for increasing disease severity. Expert consultation is recommended for guidance regarding treatment decision making. […] Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs are often used during the diagnostic phase of AOSD, but their clinical usefulness is limited. Retrospective cohort studies have shown NSAIDs to be ineffective in controlling symptoms of AOSD, with 80% of patients failing to respond. In addition, up to 20% of patients experienced an adverse event. Therefore, NSAIDs are not an effective agent in controlling disease activity. They may be used as adjunctive therapy for arthritic symptoms, but high-quality evidence for their use in this role is lacking. NSAIDs could be tried alone in a mild form of the disease for a limited period.

• #13 Adult-onset Still’s Disease Treatment Market | AoSD Treatment Market

  https://www.delveinsight.com/blog/adult-onset-stills-disease-treatment-market

  Adult-onset Stills Disease (AoSD) is a multigenic autoinflammatory disorder that stands at the junction of autoinflammatory and autoimmune diseases and involves innate and adaptive immune systems. The treatment of AoSD is currently centred on nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, and antirheumatic agents. According to older studies, NSAID as monotherapy effectively controls disease in only 715% of patients, and most patients are treated with steroids at some point in their disease course, with responses ranging from 76% to 95%, making corticosteroids the cornerstone of AoSD therapy. […] Many different therapies have been investigated in Adult-onset Stills Disease treatment market, but none has proven effective consistently. Therefore, a variety of monotherapies and combinational therapies are used to treat the affected population. The first-line therapy of AoSD consists of corticosteroid and NSAID, while the second-line therapy includes methotrexate (MTX) or cyclosporin A. In the case of refractory AoSD, the preferred option is the usage of IL-1 or IL-6 antagonists along with more biologics varying among individuals.

• #14 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Adult-onset Stills disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. […] The first part of this review deals with the treatments that are currently available for AOSD. […] Given that the current information on treatment efficacy is obtained from small retrospective case series and not from prospective, double-blind, randomized trials, the treatment of AOSD remains empirical. […] Corticosteroids remain the first-line treatment for AOSD, regardless of the clinical presentation. […] In the event of failure of corticosteroid treatment or steroid-dependence, disease-modifying anti-rheumatic drugs (DMARDS) can be considered. […] Methotrexate has proved beneficial and remains the first-line steroid-sparing treatment in AOSD.

• #15 Adult-onset Still’s Disease Treatment Market | AoSD Treatment Market

  https://www.delveinsight.com/blog/adult-onset-stills-disease-treatment-market

  Adult-onset Stills Disease (AoSD) is a multigenic autoinflammatory disorder that stands at the junction of autoinflammatory and autoimmune diseases and involves innate and adaptive immune systems. The treatment of AoSD is currently centred on nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, and antirheumatic agents. According to older studies, NSAID as monotherapy effectively controls disease in only 715% of patients, and most patients are treated with steroids at some point in their disease course, with responses ranging from 76% to 95%, making corticosteroids the cornerstone of AoSD therapy. […] Many different therapies have been investigated in Adult-onset Stills Disease treatment market, but none has proven effective consistently. Therefore, a variety of monotherapies and combinational therapies are used to treat the affected population. The first-line therapy of AoSD consists of corticosteroid and NSAID, while the second-line therapy includes methotrexate (MTX) or cyclosporin A. In the case of refractory AoSD, the preferred option is the usage of IL-1 or IL-6 antagonists along with more biologics varying among individuals.

• #16 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Glucocorticoids: Retrospective cohort studies have shown that glucocorticoids at an oral dose of 0.5 to 1 mg/kg daily of prednisone (or an equivalent dose of methylprednisolone) may be used, as they were effective in controlling AOSD symptoms in up to 65% of patients. […] Disease-modifying antirheumatic drugs (DMARDs): Adding a DMARD is critical, given the high percentage of patients who become steroid dependent. Methotrexate is the drug suggested when selecting a steroid-sparing agent. Doses of oral methotrexate range from 7.5 mg/wk to 25 mg/wk. […] Interleukin-1 (IL-1) antagonists: Three available IL-1 inhibitors anakinra, canakinumab, rilonacept have been tried in patients with AOSD. A systematic review performed in 2021 investigated the efficacy and safety of IL-1 inhibitors in AOSD, with anakinra and canakinumab recommended as treatments in patients with AOSD refractory to glucocorticoids.

• #17

  https://journals.lww.com/md-journal/fulltext/2017/03170/adult_onset_still_s_disease_with_atypical.28.aspx

  The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy with immunosuppressant drugs and/or biologic agents (mainly anakinra or tocilizumab) to control or manage symptoms because of a polycyclic or chronic course. […] The development of atypical cutaneous manifestations seems to be associated with a potentially worse prognosis, with a mortality rate reaching 8% primarily because of infectious complications related to immunosuppressive therapy. […] Recognition of this clinical variant is crucial for the early diagnosis of AOSD, as it might imply persistent disease activity and the need for more aggressive treatment. […] The great majority of patients with AOSD and atypical cutaneous lesions had persistent and severe disease, with a considerable frequency (23%) of clinical complications, including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome.

• #18 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  Recently, the identification of the key roles of IL-1 and IL-6 led to the use of bDMARDs targeting these cytokines. This has transformed the approach to patients with Stills disease, being associated with significantly improved outcomes and the reduction, if not elimination, of GC use. […] Although GCs are the cornerstone for the treatment of MAS, additional immunosuppressive and biologic treatments are more and more often used. […] In AOSD, remission with NSAIDs was not achieved in the vast majority; most patients reported side effects. […] In a recent LOR, two GC schedules in naive AOSD were compared: high (0.81mg/kg/day) versus low-dose prednisone (0.20.3mg/kg/day). At 6 months, 33/73 (45%) achieved CID, 25/38 (64.7%) in the high-dose regimen and 8/35 (22.8%) in the low-dose regimen.

• #19 Adult Still disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/diagnosis-treatment/drc-20351912

  A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects. […] Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function. […] Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes. […] The medicine methotrexate (Trexall) is often used in combination with prednisone. The prednisone dose is reduced when combined with methotrexate.

• #20 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  The goal remains to limit the use of GC in AOSD, mainly because of the side effects. IL-1 blockade interferes less during the diagnostic trajectory and can be used for a therapeutic trial. […] Severe systemic disease: either monotherapy IL-1 blockade, or GC and IL-1 blockade, or monotherapy GC. Step-up to IL-1 blockade should be considered in insufficient response on GC monotherapy or upon GC dependence. […] TNF blockade may be used in arthritis-predominant AOSD patients without systemic symptoms and upon suboptimal response to both IL-1 and IL-6 blockade.

• #21 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Adult-onset Stills disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. […] The first part of this review deals with the treatments that are currently available for AOSD. […] Given that the current information on treatment efficacy is obtained from small retrospective case series and not from prospective, double-blind, randomized trials, the treatment of AOSD remains empirical. […] Corticosteroids remain the first-line treatment for AOSD, regardless of the clinical presentation. […] In the event of failure of corticosteroid treatment or steroid-dependence, disease-modifying anti-rheumatic drugs (DMARDS) can be considered. […] Methotrexate has proved beneficial and remains the first-line steroid-sparing treatment in AOSD.

• #22 Adult Still disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/diagnosis-treatment/drc-20351912

  A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects. […] Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function. […] Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes. […] The medicine methotrexate (Trexall) is often used in combination with prednisone. The prednisone dose is reduced when combined with methotrexate.

• #23 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Adult-onset Stills disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. […] The first part of this review deals with the treatments that are currently available for AOSD. […] Given that the current information on treatment efficacy is obtained from small retrospective case series and not from prospective, double-blind, randomized trials, the treatment of AOSD remains empirical. […] Corticosteroids remain the first-line treatment for AOSD, regardless of the clinical presentation. […] In the event of failure of corticosteroid treatment or steroid-dependence, disease-modifying anti-rheumatic drugs (DMARDS) can be considered. […] Methotrexate has proved beneficial and remains the first-line steroid-sparing treatment in AOSD.

• #24 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Glucocorticoids: Retrospective cohort studies have shown that glucocorticoids at an oral dose of 0.5 to 1 mg/kg daily of prednisone (or an equivalent dose of methylprednisolone) may be used, as they were effective in controlling AOSD symptoms in up to 65% of patients. […] Disease-modifying antirheumatic drugs (DMARDs): Adding a DMARD is critical, given the high percentage of patients who become steroid dependent. Methotrexate is the drug suggested when selecting a steroid-sparing agent. Doses of oral methotrexate range from 7.5 mg/wk to 25 mg/wk. […] Interleukin-1 (IL-1) antagonists: Three available IL-1 inhibitors anakinra, canakinumab, rilonacept have been tried in patients with AOSD. A systematic review performed in 2021 investigated the efficacy and safety of IL-1 inhibitors in AOSD, with anakinra and canakinumab recommended as treatments in patients with AOSD refractory to glucocorticoids.

• #25

  https://journals.lww.com/md-journal/fulltext/2017/03170/adult_onset_still_s_disease_with_atypical.28.aspx

  Thus, most patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy consisting of immunosuppressant drugs (including methotrexate, azathioprine, cyclosporine A, and hydroxychloroquine) and/or biologic agents (mainly anakinra or tocilizumab) to control or manage symptoms because they had an intermittent/polycyclic or chronic systemic course.

• #26 ADULT ONSET STILL’S DISEASE | Comprehensive Rheumatology

  https://comprehensiverheumatology.com/index.html@p=1348.html

  Mycophenolate mofetil (Cellcept): An immunosuppressant that inhibits the proliferation of T and B lymphocytes. […] Third-line: Biologic agents like TNF-alpha inhibitors and IL-1 inhibitors may be considered in refractory cases. […] Tumor necrosis factor (TNF)-alpha inhibitors (Humira, Enbrel, Cimzia, Remicade, Simponi): Intravenous or subcutaneous medication that suppresses the physiologic response to tumor necrosis factor, which is part of the inflammatory response. […] Interleukin-1 Inhibitors (Anakinra, Ilaris): Subcutaneous medication that suppresses the physiologic response to Interleukin-1, which is part of the inflammatory response.

• #27 Adult onset Still disease

  https://dermnetnz.org/topics/adult-onset-still-disease

  There is no cure for adult-onset Still disease. The main goals of treatment are to reduce joint inflammation and avoid deformity. Therapy may include both medical and surgical interventions. In addition, the patient and their families need to fully understand the nature of the disease and how to live with it for the rest of their lives. […] Drug therapy to control many of the symptoms of Still disease includes: Aspirin, Nonsteroidal anti-inflammatory drugs such as ibuprofen, naproxen and indomethacin, Corticosteroids (e.g. prednisone), hydroxychloroquine, methotrexate, gold, azathioprine may be used for more severe symptoms, Biologics such as anakinra and antitumour necrosis factor therapies (adalimumab, etanercept, infliximab), and tocilizumab (an interleukin-6 receptor antagonist). […] Non-drug therapy includes physiotherapy, hydrotherapy and simply rest. Patients with Still disease will experience good and bad days. The number of bad days, that is flare-ups or exacerbations of the disease, can be controlled or reduced by careful planning of activities and rest periods. It has been shown that physical and emotional stress can exacerbate symptoms of Still disease.

• #28 ADULT ONSET STILL’S DISEASE | Comprehensive Rheumatology

  https://comprehensiverheumatology.com/index.html@p=1348.html

  Mycophenolate mofetil (Cellcept): An immunosuppressant that inhibits the proliferation of T and B lymphocytes. […] Third-line: Biologic agents like TNF-alpha inhibitors and IL-1 inhibitors may be considered in refractory cases. […] Tumor necrosis factor (TNF)-alpha inhibitors (Humira, Enbrel, Cimzia, Remicade, Simponi): Intravenous or subcutaneous medication that suppresses the physiologic response to tumor necrosis factor, which is part of the inflammatory response. […] Interleukin-1 Inhibitors (Anakinra, Ilaris): Subcutaneous medication that suppresses the physiologic response to Interleukin-1, which is part of the inflammatory response.

• #29 Adult-Onset Still’s Disease following Covid-19 Vaccine – A Case Report

  https://clinmedjournals.org/articles/jrdt/journal-of-rheumatic-diseases-and-treatment-jrdt-10-103.php?jid=jrdt

  The spread of COVID-19 infection has led to rapid vaccine development. It has been shown that vaccination may lead to complications like adult-onset Still’s disease. […] The diagnosis of AOSD was established based on anamnesis, radiological and laboratory testing. […] The recommended therapy was methylprednisolone with a plan to decrease the dosage to the lowest effective level and the addition of methotrexate (MTX). […] First-line treatment for AOSD is systemic corticosteroids, but in more serious and complicated cases, second-line drugs such as disease-modifying antirheumatic drugs, TNF-alpha inhibitors, and IL-1/IL-6 inhibitors can be added. […] In the presented clinical case, the patient was treated with high-dose steroids at the early stage of the disease, but as the therapy was not showing the desired results, MTX, leflunomide, and later adalimumab were added.

• #30 Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

  https://www.mdpi.com/2077-0383/10/4/733

  Adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. […] The past two decades have seen a large body of immunological research on cytokines, which has attributed to both a better understanding of AoSD and significant advances in treatment. […] The goal of our paper is to summarize the current (2020) state of knowledge on the pathogenesis, diagnosis, classification, biomarkers and complications of AoSD, as well as the treatment strategy at each stage of the disease course. […] The establishment of a default management strategy for rare diseases such as AoSD is not easy. Steroids and NSAIDs are almost always the first-line treatment regimen in both clinical phenotypes; unfortunately, they have a poor overall response. To achieve satisfactory control of the disease, many physicians offer their patients disease modifying antirheumatic drugs (DMARDs) such as methotrexate, ciclosporin or azathioprine, although there is no robust evidence to support this practice.

• #31 Adult Still disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/diagnosis-treatment/drc-20351912

  If other medicines haven’t worked, your health care provider may recommend a biologic response modifier. Biologic response modifiers are medicines that block proteins causing inflammation. These medicines are often referred to as biologics. Anakinra (Kineret), canakinumab (Ilaris) and tocilizumab (Actemra) are some biologics that are used to treat adult Still disease. Other biologics that may be helpful for treating adult Still disease include etanercept (Enbrel), infliximab (Remicade), adalimumab (Humira) and rituximab (Rituxan).

• #32 Progress in biological therapies for adult-onset Stills | BTT

  https://www.dovepress.com/progress-in-biological-therapies-for-adult-onset-stills-disease-peer-reviewed-fulltext-article-BTT

  Adult-onset Stills disease (AOSD) is a rare multifactorial autoinflammatory disorder of unknown etiology, characterized by an excessive release of cytokines triggered by dysregulated inflammation and articular and systemic manifestations. […] Nowadays, the advances in biologic agents no longer limit the treatment to NSAIDs, glucocorticoids, or conventional synthetic DMARDs. The blockade of IL-1 and IL-6 is effective in the treatment of systemic and articular inflammation of AOSD patients; however, novel compounds with different properties and targets are now available and others are being studied. […] The treatment of AOSD should be addressed to the different variety and severity of symptoms manifested. Clearly, the control of systemic inflammation and the prevention of MAS remain the main therapeutical purposes. For all severe AOSD-related complications, high-dose corticosteroids and supportive measures remain the first-line treatment. In case of inadequate response, combination with IL1 or IL-6 blockers is adequate.

• #33 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  Adult-onset Stills disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation. […] Treatment responses depended on disease phenotype: the presence of arthritis and a chronic articular phenotype were associated with a substantial response to tocilizumab with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively, whereas the systemic form and the absence of arthritis were associated with a substantial response to anakinra with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively. Tocilizumab increased the likelihood of corticosteroid withdrawal (p=0.029) regardless of delays in initiation or when it was initiated relative to other treatment in the overall therapeutic strategy.

• #34 Progress in biological therapies for adult-onset Stills | BTT

  https://www.dovepress.com/progress-in-biological-therapies-for-adult-onset-stills-disease-peer-reviewed-fulltext-article-BTT

  Nowadays, targeted biological treatments have emerged to be effective approaches for the management of AOSD, especially for the refractory form steroid-dependent or when severe manifestations occur. […] Anti IL-1 agents undoubtedly represent the milestone for the treatment of AOSD. […] IL-1 inhibitors have an overall satisfactory safety profile in AOSD. In terms of the risk of infection, treatment with anti-IL-1 agents seems to have an acceptable safety profile. […] The efficacy of TCZ was proved in cases of refractory AOSD, especially when the chronic articular pattern was predominant. […] Recent advances in biologic drug development have had a major impact on AOSD patients in terms of improved quality-of-life and coping strategies. Specific inhibition of IL-1 and IL-6 is now considered a safe and effective therapy to better control the disease.

• #35 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Data concerning intravenous immunoglobulin are more controversial, with two randomized open-label trials showing some efficacy when used early in the course of AOSD, whereas retrospective data do not support the efficacy of intravenous immunoglobulin in AOSD. […] Although dramatic responses have been reported in systemic-onset JIA when given as first-line treatment, targeted biologic agents are actually reserved for refractory AOSD. […] Anakinra has been more frequently reported in the treatment of AOSD. […] Tocilizumab should be considered as an alternative to IL-1 antagonists, particularly when articular signs such as joint erosion accompany systemic symptoms. […] In patients with articular refractory AOSD, anti-TNF- should be the preferred treatment. […] Finally, it should be noted that, in an effort to standardize therapeutic management and evaluate comparative effectiveness in an observational setting, the Childhood Arthritis and Rheumatology Research Alliance has developed four consensus treatment plans for systemic-onset JIA.

• #36

  https://link.springer.com/article/10.1007/s40265-024-01993-x

  Currently, the anti-cytokines employed for the treatment of AOSD target IL-1, IL-6 and TNF-alpha, which are the pivotal cytokines involved in AOSD pathogenesis. […] Anakinra has a short half-life and is usually administered at the posology of 100 mg/day (12 mg/kg/day in children) subcutaneously (SC), but higher dosages may be required if clinical manifestations are not sufficiently controlled. […] Canakinumab is a fully human monoclonal antibody targeting IL-1. It is approved by EMA for sJIA/AOSD treatment, and is normally administered subcutaneously at a dosage of 150 mg or 300 mg/4 weeks. […] IL-6 is another central cytokine involved in AOSD pathogenesis and is often associated with articular manifestations. Tocilizumab competitively inhibits the binding of IL-6 to its receptor (IL-6R), and it is currently approved for the treatment of refractory sJIA and in patients older than 2 years of age. […] Overall, many compounds are under study and evaluation in in vivo or in vitro models. Despite most of the studies currently being at an early stage, it is possible to conceive their future employment in refractory AOSD.

• #37 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  ANK is generally administered subcutaneously. […] ANK has been evaluated in two SRs, two RCTs and 28 LOP/LOR studies. […] In a double-blind RCT on 24 patients with sJIA, ANK (2 mg/kg/day, maximum 100 mg/day) was compared with placebo. […] CAM is a fully human monoclonal antibody against IL-1, approved for sJIA in 2013, and for AOSD in 2020. […] In a pooled analysis conducted in AOSD, CID was achieved in 69% of the 99 patients. […] In a randomised withdrawal-design trial in sJIA, the CID rate was 31% during the open-label lead-in phase. […] The most common AEs with ANK were injection site reactions (ISR) reported with variable frequencies. […] The most frequent AEs reported with CAM were infections, gastrointestinal disorders, skin/subcutaneous disorders and cytopenia. […] A MAS rate of 2.8/100 PY was calculated based on data from two clinical trials (a total of 324 patients). […] The current systematic review (SR) aimed to gather scientific evidence on the efficacy and safety of treatments for sJIA/AOSD and MAS.

• #38 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  ANK is generally administered subcutaneously. […] ANK has been evaluated in two SRs, two RCTs and 28 LOP/LOR studies. […] In a double-blind RCT on 24 patients with sJIA, ANK (2 mg/kg/day, maximum 100 mg/day) was compared with placebo. […] CAM is a fully human monoclonal antibody against IL-1, approved for sJIA in 2013, and for AOSD in 2020. […] In a pooled analysis conducted in AOSD, CID was achieved in 69% of the 99 patients. […] In a randomised withdrawal-design trial in sJIA, the CID rate was 31% during the open-label lead-in phase. […] The most common AEs with ANK were injection site reactions (ISR) reported with variable frequencies. […] The most frequent AEs reported with CAM were infections, gastrointestinal disorders, skin/subcutaneous disorders and cytopenia. […] A MAS rate of 2.8/100 PY was calculated based on data from two clinical trials (a total of 324 patients). […] The current systematic review (SR) aimed to gather scientific evidence on the efficacy and safety of treatments for sJIA/AOSD and MAS.

• #39

  https://link.springer.com/article/10.1007/s40265-024-01993-x

  Currently, the anti-cytokines employed for the treatment of AOSD target IL-1, IL-6 and TNF-alpha, which are the pivotal cytokines involved in AOSD pathogenesis. […] Anakinra has a short half-life and is usually administered at the posology of 100 mg/day (12 mg/kg/day in children) subcutaneously (SC), but higher dosages may be required if clinical manifestations are not sufficiently controlled. […] Canakinumab is a fully human monoclonal antibody targeting IL-1. It is approved by EMA for sJIA/AOSD treatment, and is normally administered subcutaneously at a dosage of 150 mg or 300 mg/4 weeks. […] IL-6 is another central cytokine involved in AOSD pathogenesis and is often associated with articular manifestations. Tocilizumab competitively inhibits the binding of IL-6 to its receptor (IL-6R), and it is currently approved for the treatment of refractory sJIA and in patients older than 2 years of age. […] Overall, many compounds are under study and evaluation in in vivo or in vitro models. Despite most of the studies currently being at an early stage, it is possible to conceive their future employment in refractory AOSD.

• #40 FDA Approves First Treatment for Adult Onset Still’s Disease, a Severe and Rare Disease | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-adult-onset-stills-disease-severe-and-rare-disease

  The U.S. Food and Drug Administration today approved Ilaris (canakinumab) injection for the treatment of Active Stills disease, including Adult-Onset Stills Disease (AOSD). […] Prior to todays approval, patients had no FDA-approved treatments for their disease, which can include symptoms such as painful arthritis, fevers and rash, said Nikolay Nikolov, M.D., acting director of the Division of Rheumatology and Transplant Medicine in the FDAs Center for Drug Evaluation and Research. Todays approval provides patients with a treatment option. […] Ilaris works by blocking the effects of IL-1 and suppressing inflammation in patients with this autoinflammatory disorder. […] Common side effects reported by patients treated with Ilaris are infections (colds and upper respiratory tract infections), abdominal pain and injection-site reactions. The prescribing information for Ilaris includes a warning for potential increased risk of serious infections due to IL-1 blockade.

• #41 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  ANK is generally administered subcutaneously. […] ANK has been evaluated in two SRs, two RCTs and 28 LOP/LOR studies. […] In a double-blind RCT on 24 patients with sJIA, ANK (2 mg/kg/day, maximum 100 mg/day) was compared with placebo. […] CAM is a fully human monoclonal antibody against IL-1, approved for sJIA in 2013, and for AOSD in 2020. […] In a pooled analysis conducted in AOSD, CID was achieved in 69% of the 99 patients. […] In a randomised withdrawal-design trial in sJIA, the CID rate was 31% during the open-label lead-in phase. […] The most common AEs with ANK were injection site reactions (ISR) reported with variable frequencies. […] The most frequent AEs reported with CAM were infections, gastrointestinal disorders, skin/subcutaneous disorders and cytopenia. […] A MAS rate of 2.8/100 PY was calculated based on data from two clinical trials (a total of 324 patients). […] The current systematic review (SR) aimed to gather scientific evidence on the efficacy and safety of treatments for sJIA/AOSD and MAS.

• #42 FDA Approves First Treatment for Adult Onset Still’s Disease, a Severe and Rare Disease | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-adult-onset-stills-disease-severe-and-rare-disease

  The U.S. Food and Drug Administration today approved Ilaris (canakinumab) injection for the treatment of Active Stills disease, including Adult-Onset Stills Disease (AOSD). […] Prior to todays approval, patients had no FDA-approved treatments for their disease, which can include symptoms such as painful arthritis, fevers and rash, said Nikolay Nikolov, M.D., acting director of the Division of Rheumatology and Transplant Medicine in the FDAs Center for Drug Evaluation and Research. Todays approval provides patients with a treatment option. […] Ilaris works by blocking the effects of IL-1 and suppressing inflammation in patients with this autoinflammatory disorder. […] Common side effects reported by patients treated with Ilaris are infections (colds and upper respiratory tract infections), abdominal pain and injection-site reactions. The prescribing information for Ilaris includes a warning for potential increased risk of serious infections due to IL-1 blockade.

• #43 Adult-Onset Still’s Disease—A Complex Disease, a Challenging Treatment

  https://www.mdpi.com/1422-0067/23/21/12810

  There are currently three IL-1 inhibitors available for AOSD: Anakinra (an IL-1R antagonist), canakinumab (an anti-IL-1 monoclonal antibody), and rilonacept (a soluble IL-1 trap molecule). […] Tocilizumab was linked to a notable improvement in systemic and articular clinical symptoms, a significant steroid-sparing benefit, and an acceptable safety profile in this research. […] The most frequent AEs are mild infections, injection-site reactions, neutropenia, and hepatotoxicity. […] In AOSD, unresponsive to conventional therapy and other biologics, the suppression of IL-1 is an effective therapeutic strategy.

• #44 Adult-onset Still’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Adult-onset_Still%27s_disease

  In June 2020 FDA approved Ilaris (canakinumab) for the treatment of AOSD, this is the first FDA approved treatment for AOSD. Canakinumab is another anti-IL1 drug which selectively binds IL-1 and rilonacept which blocks both IL-1A and IL-1. The monoclonal anti-IL6 antibody tocilizumab is another treatment option as effective as anakinra.

• #45 Progress in biological therapies for adult-onset Stills | BTT

  https://www.dovepress.com/progress-in-biological-therapies-for-adult-onset-stills-disease-peer-reviewed-fulltext-article-BTT

  Nowadays, targeted biological treatments have emerged to be effective approaches for the management of AOSD, especially for the refractory form steroid-dependent or when severe manifestations occur. […] Anti IL-1 agents undoubtedly represent the milestone for the treatment of AOSD. […] IL-1 inhibitors have an overall satisfactory safety profile in AOSD. In terms of the risk of infection, treatment with anti-IL-1 agents seems to have an acceptable safety profile. […] The efficacy of TCZ was proved in cases of refractory AOSD, especially when the chronic articular pattern was predominant. […] Recent advances in biologic drug development have had a major impact on AOSD patients in terms of improved quality-of-life and coping strategies. Specific inhibition of IL-1 and IL-6 is now considered a safe and effective therapy to better control the disease.

• #46 FDA Approves First Treatment for Adult Onset Still’s Disease, a Severe and Rare Disease | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-adult-onset-stills-disease-severe-and-rare-disease

  The U.S. Food and Drug Administration today approved Ilaris (canakinumab) injection for the treatment of Active Stills disease, including Adult-Onset Stills Disease (AOSD). […] Prior to todays approval, patients had no FDA-approved treatments for their disease, which can include symptoms such as painful arthritis, fevers and rash, said Nikolay Nikolov, M.D., acting director of the Division of Rheumatology and Transplant Medicine in the FDAs Center for Drug Evaluation and Research. Todays approval provides patients with a treatment option. […] Ilaris works by blocking the effects of IL-1 and suppressing inflammation in patients with this autoinflammatory disorder. […] Common side effects reported by patients treated with Ilaris are infections (colds and upper respiratory tract infections), abdominal pain and injection-site reactions. The prescribing information for Ilaris includes a warning for potential increased risk of serious infections due to IL-1 blockade.

• #47 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  Adult-onset Stills disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation. […] Treatment responses depended on disease phenotype: the presence of arthritis and a chronic articular phenotype were associated with a substantial response to tocilizumab with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively, whereas the systemic form and the absence of arthritis were associated with a substantial response to anakinra with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively. Tocilizumab increased the likelihood of corticosteroid withdrawal (p=0.029) regardless of delays in initiation or when it was initiated relative to other treatment in the overall therapeutic strategy.

• #48

  https://link.springer.com/article/10.1007/s00296-020-04622-4

  Adult-onset Still’s disease (AOSD) is defined as a systemic inflammatory disorder of unknown aetiology and is classified as a multigene autoinflammatory disease. Treatment of AOSD still remains mostly empirical with nonsteroidal anti-inflammatory drugs, glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs or cyclosporin A. Inhibitors of tumour necrosis factor-alpha and interleukin-1 (IL-1) antagonists have shown efficacy in certain subsets of patients with AOSD. The IL-6 molecule is one of the potential targets in treating AOSD considering that its level is increased in both the systemic and chronic articular forms of the disease. […] We present a series of eight patients from our centre with refractory AOSD treated with tocilizumab (TCZ). The drug was administered intravenously (68 mg/kg every 34 weeks) or subcutaneously (162 mg weekly). One patient had a disease relapse during TCZ therapy, and the drug had to be withdrawn in one patient due to a severe infection, while five out of six patients currently treated are in stable remission.

• #49 Adult-Onset Still’s Disease—A Complex Disease, a Challenging Treatment

  https://www.mdpi.com/1422-0067/23/21/12810

  There are currently three IL-1 inhibitors available for AOSD: Anakinra (an IL-1R antagonist), canakinumab (an anti-IL-1 monoclonal antibody), and rilonacept (a soluble IL-1 trap molecule). […] Tocilizumab was linked to a notable improvement in systemic and articular clinical symptoms, a significant steroid-sparing benefit, and an acceptable safety profile in this research. […] The most frequent AEs are mild infections, injection-site reactions, neutropenia, and hepatotoxicity. […] In AOSD, unresponsive to conventional therapy and other biologics, the suppression of IL-1 is an effective therapeutic strategy.

• #50 Adult-Onset Still’s Disease—A Complex Disease, a Challenging Treatment

  https://www.mdpi.com/1422-0067/23/21/12810

  There are currently three IL-1 inhibitors available for AOSD: Anakinra (an IL-1R antagonist), canakinumab (an anti-IL-1 monoclonal antibody), and rilonacept (a soluble IL-1 trap molecule). […] Tocilizumab was linked to a notable improvement in systemic and articular clinical symptoms, a significant steroid-sparing benefit, and an acceptable safety profile in this research. […] The most frequent AEs are mild infections, injection-site reactions, neutropenia, and hepatotoxicity. […] In AOSD, unresponsive to conventional therapy and other biologics, the suppression of IL-1 is an effective therapeutic strategy.

• #51 Successful Treatment of Recurrent Adult-Onset Still’s Disease wi | CCID

  https://www.dovepress.com/successful-treatment-of-recurrent-adult-onset-stills-disease-with-toci-peer-reviewed-fulltext-article-CCID

  As shown in this case, TCZ can rapidly control the symptoms of skin lesions, joint and muscle pain, and sore throat in adult patients with recurrent AOSD, effectively reduce all inflammatory indexes, and facilitate the reduction of glucocorticoid dose. […] However, the US Food and Drug Administration has not yet approved TCZ as a therapeutic drug for AOSD. […] Therefore, more multicenter collaborative research is needed as a basis to enhance the persuasiveness of TCZ as a treatment choice for AOSD patients, especially in low- and middle-income countries and regions. […] In addition, TCZ can also be used in refractory AOSD patients with macrophage activation syndrome. […] Consequently, extensive randomized controlled trials are essential in future work to further substantiate the efficacy and safety of TCZ in AOSD treatment, as well as to establish treatment guidelines.

• #52 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  TNF-alpha inhibitors: Three available TNF-alpha inhibitors infliximab, etanercept, and adalimumab have been tried in patients with AOSD. […] Interleukin-6 (IL-6) antagonist: Tocilizumab is a fully humanized monoclonal antibody against IL-6 receptors, which was used in 1 prospective open-label trial of 14 patients with AOSD (who previously failed anakinra and 1 TNF-alpha inhibitor). […] Janus kinase (JAK) inhibitors: There is limited evidence from case series on the use of JAK inhibitors in patients with AOSD refractory to conventional therapies. […] IV immunoglobulins: Limited evidence from case reports and case series supports some benefit of IV immunoglobulin infusions in patients resistant to or unable to tolerate other forms of therapy. […] The use of an IL-18-binding protein and IL-6 antagonist sarilumab has been described in the literature in refractory cases.

• #53 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  Adult-onset Stills disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation. […] Treatment responses depended on disease phenotype: the presence of arthritis and a chronic articular phenotype were associated with a substantial response to tocilizumab with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively, whereas the systemic form and the absence of arthritis were associated with a substantial response to anakinra with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively. Tocilizumab increased the likelihood of corticosteroid withdrawal (p=0.029) regardless of delays in initiation or when it was initiated relative to other treatment in the overall therapeutic strategy.

• #54 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Data concerning intravenous immunoglobulin are more controversial, with two randomized open-label trials showing some efficacy when used early in the course of AOSD, whereas retrospective data do not support the efficacy of intravenous immunoglobulin in AOSD. […] Although dramatic responses have been reported in systemic-onset JIA when given as first-line treatment, targeted biologic agents are actually reserved for refractory AOSD. […] Anakinra has been more frequently reported in the treatment of AOSD. […] Tocilizumab should be considered as an alternative to IL-1 antagonists, particularly when articular signs such as joint erosion accompany systemic symptoms. […] In patients with articular refractory AOSD, anti-TNF- should be the preferred treatment. […] Finally, it should be noted that, in an effort to standardize therapeutic management and evaluate comparative effectiveness in an observational setting, the Childhood Arthritis and Rheumatology Research Alliance has developed four consensus treatment plans for systemic-onset JIA.

• #55 Successful Treatment of Recurrent Adult-Onset Still’s Disease wi | CCID

  https://www.dovepress.com/successful-treatment-of-recurrent-adult-onset-stills-disease-with-toci-peer-reviewed-fulltext-article-CCID

  In cases of recurrent refractory episodes or severe life-threatening systemic symptoms in AOSD, the utilization of biologic agents may be considered for treatment. […] Various categories of biologic agents are available for the management of AOSD, including TNF- inhibitors, IL-1 inhibitors, IL-6 inhibitors, and other biologics. […] Among these, TNF- inhibitors were the earliest biologic agents employed in AOSD treatment and have demonstrated efficacy in patients with chronic arthritis. […] A retrospective study comparing the effectiveness of different biologic agents in treating AOSD revealed that TNF- inhibitors exhibited lower efficacy in compared to IL-1 inhibitors and IL-6 inhibitors, suggesting a potential requirement for switching to the other two biologics to sustain disease remission.

• #56 Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

  https://www.mdpi.com/2077-0383/10/4/733

  In contrast to numerous trials in the field of rheumatoid arthritis and spondyloarthritis, the efficacy of TNFα blockers in AoSD is controversial. […] Anakinra, a recombinant humanized IL-1 receptor antagonist, is the first choice for AoSD, yet patients with mainly articular phenotypes do not always benefit. […] The other strategy for inhibiting IL-1 that has been intensively studied to date consists of a fully human antibody against IL-1β, canakinumab. […] Tocilizumab, a humanized monoclonal antibody against the IL-6 receptor, showed promising results in the treatment of AoSD in a pilot study. […] Janus kinase (JAK) inhibitors block a wide variety of proinflammatory cells and can therefore become a very promising treatment approach in heterogeneous disorders, such as AoSD. […] Given the new insights into the pathogenic role of IL-18 in AoSD, this cytokine quickly became a drug target.

• #57 Successful Treatment of Recurrent Adult-Onset Still’s Disease wi | CCID

  https://www.dovepress.com/successful-treatment-of-recurrent-adult-onset-stills-disease-with-toci-peer-reviewed-fulltext-article-CCID

  In cases of recurrent refractory episodes or severe life-threatening systemic symptoms in AOSD, the utilization of biologic agents may be considered for treatment. […] Various categories of biologic agents are available for the management of AOSD, including TNF- inhibitors, IL-1 inhibitors, IL-6 inhibitors, and other biologics. […] Among these, TNF- inhibitors were the earliest biologic agents employed in AOSD treatment and have demonstrated efficacy in patients with chronic arthritis. […] A retrospective study comparing the effectiveness of different biologic agents in treating AOSD revealed that TNF- inhibitors exhibited lower efficacy in compared to IL-1 inhibitors and IL-6 inhibitors, suggesting a potential requirement for switching to the other two biologics to sustain disease remission.

• #58 Successful Treatment with Etanercept in a Patient with Adult-onset Still’s Disease

  https://www.jrd.or.kr/journal/view.html?uid=592&vmd=Full

  Successful Treatment with Etanercept in a Patient with Adult-onset Still’s Disease. Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. Non-steroidal anti-inflammatory drugs (NSAIDs), steroids, disease modifying anti-rheumatic drugs (DMARDs), immunosuppressives and intravenous immunoglobulin (IVIG) have been used to control the disease. Recent reports showed the clinical effectiveness of TNF-α blockers (infliximab and etanercept) in refractory AOSD. We report a case successfully treated with etanercept in the early AOSD refractory to the combination therapy of high-dose prednisolone and cyclosporine (CSA). […] Keywords: Adult onset Still’s disease, Etanercept, Tumor necrosis factor-α.

• #59 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  The goal remains to limit the use of GC in AOSD, mainly because of the side effects. IL-1 blockade interferes less during the diagnostic trajectory and can be used for a therapeutic trial. […] Severe systemic disease: either monotherapy IL-1 blockade, or GC and IL-1 blockade, or monotherapy GC. Step-up to IL-1 blockade should be considered in insufficient response on GC monotherapy or upon GC dependence. […] TNF blockade may be used in arthritis-predominant AOSD patients without systemic symptoms and upon suboptimal response to both IL-1 and IL-6 blockade.

• #60

  https://www.reumatismo.org/index.php/reuma/article/view/888

  Adult-onset Stills disease (AOSD) is an uncommon inflammatory condition of unknown origin. […] Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising. We report here the case of a 38-year-old female patient with AOSD refractory to cytotoxic agents, treated by rituximab infusion therapy with favorable outcome.

• #61 Adult Still disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/diagnosis-treatment/drc-20351912

  If other medicines haven’t worked, your health care provider may recommend a biologic response modifier. Biologic response modifiers are medicines that block proteins causing inflammation. These medicines are often referred to as biologics. Anakinra (Kineret), canakinumab (Ilaris) and tocilizumab (Actemra) are some biologics that are used to treat adult Still disease. Other biologics that may be helpful for treating adult Still disease include etanercept (Enbrel), infliximab (Remicade), adalimumab (Humira) and rituximab (Rituxan).

• #62 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  TNF-alpha inhibitors: Three available TNF-alpha inhibitors infliximab, etanercept, and adalimumab have been tried in patients with AOSD. […] Interleukin-6 (IL-6) antagonist: Tocilizumab is a fully humanized monoclonal antibody against IL-6 receptors, which was used in 1 prospective open-label trial of 14 patients with AOSD (who previously failed anakinra and 1 TNF-alpha inhibitor). […] Janus kinase (JAK) inhibitors: There is limited evidence from case series on the use of JAK inhibitors in patients with AOSD refractory to conventional therapies. […] IV immunoglobulins: Limited evidence from case reports and case series supports some benefit of IV immunoglobulin infusions in patients resistant to or unable to tolerate other forms of therapy. […] The use of an IL-18-binding protein and IL-6 antagonist sarilumab has been described in the literature in refractory cases.

• #63 Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

  https://www.mdpi.com/2077-0383/10/4/733

  In contrast to numerous trials in the field of rheumatoid arthritis and spondyloarthritis, the efficacy of TNFα blockers in AoSD is controversial. […] Anakinra, a recombinant humanized IL-1 receptor antagonist, is the first choice for AoSD, yet patients with mainly articular phenotypes do not always benefit. […] The other strategy for inhibiting IL-1 that has been intensively studied to date consists of a fully human antibody against IL-1β, canakinumab. […] Tocilizumab, a humanized monoclonal antibody against the IL-6 receptor, showed promising results in the treatment of AoSD in a pilot study. […] Janus kinase (JAK) inhibitors block a wide variety of proinflammatory cells and can therefore become a very promising treatment approach in heterogeneous disorders, such as AoSD. […] Given the new insights into the pathogenic role of IL-18 in AoSD, this cytokine quickly became a drug target.

• #64 Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

  https://www.mdpi.com/2077-0383/10/4/733

  In contrast to numerous trials in the field of rheumatoid arthritis and spondyloarthritis, the efficacy of TNFα blockers in AoSD is controversial. […] Anakinra, a recombinant humanized IL-1 receptor antagonist, is the first choice for AoSD, yet patients with mainly articular phenotypes do not always benefit. […] The other strategy for inhibiting IL-1 that has been intensively studied to date consists of a fully human antibody against IL-1β, canakinumab. […] Tocilizumab, a humanized monoclonal antibody against the IL-6 receptor, showed promising results in the treatment of AoSD in a pilot study. […] Janus kinase (JAK) inhibitors block a wide variety of proinflammatory cells and can therefore become a very promising treatment approach in heterogeneous disorders, such as AoSD. […] Given the new insights into the pathogenic role of IL-18 in AoSD, this cytokine quickly became a drug target.

• #65

  https://link.springer.com/article/10.1007/s40265-024-01993-x

  Currently, the anti-cytokines employed for the treatment of AOSD target IL-1, IL-6 and TNF-alpha, which are the pivotal cytokines involved in AOSD pathogenesis. […] Anakinra has a short half-life and is usually administered at the posology of 100 mg/day (12 mg/kg/day in children) subcutaneously (SC), but higher dosages may be required if clinical manifestations are not sufficiently controlled. […] Canakinumab is a fully human monoclonal antibody targeting IL-1. It is approved by EMA for sJIA/AOSD treatment, and is normally administered subcutaneously at a dosage of 150 mg or 300 mg/4 weeks. […] IL-6 is another central cytokine involved in AOSD pathogenesis and is often associated with articular manifestations. Tocilizumab competitively inhibits the binding of IL-6 to its receptor (IL-6R), and it is currently approved for the treatment of refractory sJIA and in patients older than 2 years of age. […] Overall, many compounds are under study and evaluation in in vivo or in vitro models. Despite most of the studies currently being at an early stage, it is possible to conceive their future employment in refractory AOSD.

• #66 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  This study highlights the therapeutic implications of the phenotypic dichotomy of AOSD and should help us better codify AOSD treatment. […] The presence of arthritis and a chronic articular phenotype may be associated with a preferential response to tocilizumab, whereas the systemic form may be associated with a preferential response to anakinra. […] Tocilizumab increases the likelihood of corticosteroid withdrawal regardless of delays in its initiation or when it is initiated relative to other treatment in the overall therapeutic strategy. […] These results should help us better codify the treatment strategy for adult-onset Stills disease. […] This study supports the dichotomy of AOSD in two phenotypes and introduces the idea that treatment response to bDMARD may depend on disease phenotype. The presence of arthritis and a chronic articular phenotype seem to be associated with a substantial response to tocilizumab, whereas the systemic form seems to be associated with a substantial response to anakinra.

• #67 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  Adult-onset Stills disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation. […] Treatment responses depended on disease phenotype: the presence of arthritis and a chronic articular phenotype were associated with a substantial response to tocilizumab with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively, whereas the systemic form and the absence of arthritis were associated with a substantial response to anakinra with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively. Tocilizumab increased the likelihood of corticosteroid withdrawal (p=0.029) regardless of delays in initiation or when it was initiated relative to other treatment in the overall therapeutic strategy.

• #68 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  The goal remains to limit the use of GC in AOSD, mainly because of the side effects. IL-1 blockade interferes less during the diagnostic trajectory and can be used for a therapeutic trial. […] Severe systemic disease: either monotherapy IL-1 blockade, or GC and IL-1 blockade, or monotherapy GC. Step-up to IL-1 blockade should be considered in insufficient response on GC monotherapy or upon GC dependence. […] TNF blockade may be used in arthritis-predominant AOSD patients without systemic symptoms and upon suboptimal response to both IL-1 and IL-6 blockade.

• #69 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  Objectives To analyse the efficacy and safety of treatments for Stills disease and macrophage activation syndrome (MAS). […] IL-1i and IL-6Ri show the highest level of efficacy in the treatment of Stills disease. For MAS, IL-1 and interferon- inhibition appear to be effective on a background of high-dose glucocorticoids. […] Interleukin-1 inhibitors (IL-1i) and IL-6Ri show the highest level of evidence in terms of efficacy, safety and an acceptable risk-benefit ratio for the treatment of sJIA and AOSD. […] IL-1 and IL-6R inhibitors show the most interesting risk-benefit ratio compared with all alternatives in Stills disease. In MAS, despite the scarcity of data, high-dose glucocorticoids combined with IL-1 or IFN- inhibition appear presently to be the best available strategy. […] The therapeutic approach in both sJIA and AOSD historically relies on non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids (GCs).

• #70 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  Adult-onset Stills disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation. […] Treatment responses depended on disease phenotype: the presence of arthritis and a chronic articular phenotype were associated with a substantial response to tocilizumab with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively, whereas the systemic form and the absence of arthritis were associated with a substantial response to anakinra with p=0.0009 (OR 36 [2.61703]) and p=0.017 (OR 10 [1.2292.6]), respectively. Tocilizumab increased the likelihood of corticosteroid withdrawal (p=0.029) regardless of delays in initiation or when it was initiated relative to other treatment in the overall therapeutic strategy.

• #71 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Data concerning intravenous immunoglobulin are more controversial, with two randomized open-label trials showing some efficacy when used early in the course of AOSD, whereas retrospective data do not support the efficacy of intravenous immunoglobulin in AOSD. […] Although dramatic responses have been reported in systemic-onset JIA when given as first-line treatment, targeted biologic agents are actually reserved for refractory AOSD. […] Anakinra has been more frequently reported in the treatment of AOSD. […] Tocilizumab should be considered as an alternative to IL-1 antagonists, particularly when articular signs such as joint erosion accompany systemic symptoms. […] In patients with articular refractory AOSD, anti-TNF- should be the preferred treatment. […] Finally, it should be noted that, in an effort to standardize therapeutic management and evaluate comparative effectiveness in an observational setting, the Childhood Arthritis and Rheumatology Research Alliance has developed four consensus treatment plans for systemic-onset JIA.

• #72 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  The goal remains to limit the use of GC in AOSD, mainly because of the side effects. IL-1 blockade interferes less during the diagnostic trajectory and can be used for a therapeutic trial. […] Severe systemic disease: either monotherapy IL-1 blockade, or GC and IL-1 blockade, or monotherapy GC. Step-up to IL-1 blockade should be considered in insufficient response on GC monotherapy or upon GC dependence. […] TNF blockade may be used in arthritis-predominant AOSD patients without systemic symptoms and upon suboptimal response to both IL-1 and IL-6 blockade.

• #73 Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-1838-6

  This study highlights the therapeutic implications of the phenotypic dichotomy of AOSD and should help us better codify AOSD treatment. […] The presence of arthritis and a chronic articular phenotype may be associated with a preferential response to tocilizumab, whereas the systemic form may be associated with a preferential response to anakinra. […] Tocilizumab increases the likelihood of corticosteroid withdrawal regardless of delays in its initiation or when it is initiated relative to other treatment in the overall therapeutic strategy. […] These results should help us better codify the treatment strategy for adult-onset Stills disease. […] This study supports the dichotomy of AOSD in two phenotypes and introduces the idea that treatment response to bDMARD may depend on disease phenotype. The presence of arthritis and a chronic articular phenotype seem to be associated with a substantial response to tocilizumab, whereas the systemic form seems to be associated with a substantial response to anakinra.

• #74 Adult Still’s Disease

  https://www.arthritis.org/diseases/adult-stills-disease

  Doctors use several drugs to treat adult Still’s disease. Over-the-counter or prescription nonsteroidal anti-inflammatory drugs (NSAIDs) help to reduce mild pain and inflammation. Corticosteroids, such as prednisone, are needed if the disease is severe or doesn’t respond to prescription NSAIDs. Disease-modifying drugs (DMARDs), such as methotrexate, and biologics, are needed in more severe cases or if the arthritis becomes chronic. It may be necessary to take more than one medication at a time to control symptoms. […] With adult Stills disease, the medications may need to be taken even after symptoms go away. This is called maintenance therapy. Its important to keep inflammation under control to prevent damage to joints and organs.

• #75 Adult Still disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/diagnosis-treatment/drc-20351912

  A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects. […] Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function. […] Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes. […] The medicine methotrexate (Trexall) is often used in combination with prednisone. The prednisone dose is reduced when combined with methotrexate.

• #76 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  The goal remains to limit the use of GC in AOSD, mainly because of the side effects. IL-1 blockade interferes less during the diagnostic trajectory and can be used for a therapeutic trial. […] Severe systemic disease: either monotherapy IL-1 blockade, or GC and IL-1 blockade, or monotherapy GC. Step-up to IL-1 blockade should be considered in insufficient response on GC monotherapy or upon GC dependence. […] TNF blockade may be used in arthritis-predominant AOSD patients without systemic symptoms and upon suboptimal response to both IL-1 and IL-6 blockade.

• #77 Adult-onset Still’s disease | Symptoms, treatments and new research

  https://versusarthritis.org/about-arthritis/conditions/adult-onset-still-s-disease/

  DMARDs can be used to reduce the effects of the immune system attacking your own body. […] Biological therapies are newer drugs to treat inflammatory types of arthritis, such as AOSD. They block certain chemicals or cells in your immune system to reduce the activity of your condition. […] You may need to take a biological therapy alongside another DMARD, usually methotrexate, to fully bring the condition under control.

• #78 Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-2021-9

  Adult-onset Stills disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. There was a large consensus that failure of the first IL-1 inhibitor does not preclude response to another one. IL-1 inhibition represents an effective therapeutic approach in AOSD refractory to conventional treatment and/or other biologics. In AOSD, treatment with IL-1 Inhibitors is effective on different clinical and laboratory parameters and displays a significant steroid-sparing effect in most patients. IL-1 inhibitors are effective in the treatment of AOSD-related Macrophage Activation Syndrome (MAS). IL-1 Inhibitors have an overall satisfactory safety profile in AOSD. Based on our review of the literature, therapies targeting IL-1 (anakinra, canakinumab, and rilonacept) are significantly effective in patients with AOSD refractory to conventional treatment. The effectiveness of anti-IL-1 therapies in AOSD ranged from 50 to 100%. Data on the efficacy of anti-IL1 agents in the treatment of arthritis in patients with AOSD are still controversial. A different profile of efficacy among IL-1 inhibitors cannot be established, because head-to-head comparisons are lacking. Indirect data show that IL-1 inhibitors can be effective in AOSD, both in first and subsequent lines of biologic treatment. Some data suggest that IL-1 inhibitors may be more effective on systemic rather than chronic articular manifestations of AOSD.

• #79 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Data concerning intravenous immunoglobulin are more controversial, with two randomized open-label trials showing some efficacy when used early in the course of AOSD, whereas retrospective data do not support the efficacy of intravenous immunoglobulin in AOSD. […] Although dramatic responses have been reported in systemic-onset JIA when given as first-line treatment, targeted biologic agents are actually reserved for refractory AOSD. […] Anakinra has been more frequently reported in the treatment of AOSD. […] Tocilizumab should be considered as an alternative to IL-1 antagonists, particularly when articular signs such as joint erosion accompany systemic symptoms. […] In patients with articular refractory AOSD, anti-TNF- should be the preferred treatment. […] Finally, it should be noted that, in an effort to standardize therapeutic management and evaluate comparative effectiveness in an observational setting, the Childhood Arthritis and Rheumatology Research Alliance has developed four consensus treatment plans for systemic-onset JIA.

• #80 Adult Still’s Disease

  https://www.arthritis.org/diseases/adult-stills-disease

  Doctors use several drugs to treat adult Still’s disease. Over-the-counter or prescription nonsteroidal anti-inflammatory drugs (NSAIDs) help to reduce mild pain and inflammation. Corticosteroids, such as prednisone, are needed if the disease is severe or doesn’t respond to prescription NSAIDs. Disease-modifying drugs (DMARDs), such as methotrexate, and biologics, are needed in more severe cases or if the arthritis becomes chronic. It may be necessary to take more than one medication at a time to control symptoms. […] With adult Stills disease, the medications may need to be taken even after symptoms go away. This is called maintenance therapy. Its important to keep inflammation under control to prevent damage to joints and organs.

• #81 Still’s disease: Causes, symptoms, and treatment

  https://www.medicalnewstoday.com/articles/stills-disease

  The aims of treatment are: […] Doctors may prescribe or recommend various medications to treat AOSD. These may include: […] A person may still need to take these medications even after the symptoms go away. This maintenance therapy can keep inflammation under control and prevent further bone, cartilage, and organ damage. […] A person should talk with a doctor to identify the best treatment and management plan for their symptoms.

• #82 EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1614

  The TF emphasises that the presently available evidence is not sufficient to withdraw efficacious therapies with IL-1 or IL-6 inhibitors, considering the fact that their withdrawal is not associated with significant improvement in most patients, that there is a significant risk for severe flares, often with MAS, and the burden of long-term high-dose GCs that represents the only alternative for many of these patients.

• #83 Biological treatment in resistant adult-onset Still’s disease: A single-center, retrospective cohort study | Volume 37 – Issue 1 – March 2022 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/1304

  Biological drugs seem to be effective for AOSD patients who are resistant to conventional therapies. Due to the administration methods and the high costs of these drugs, however, tapering the treatment should be considered, after remission is achieved. […] Biological drugs may be rarely required for patients with active disease and arthritis resistant to conventional therapies. However, many cytokines play a role in the pathogenesis of the disease, inhibition of the main cytokines with biological drugs is crucial. Using IL-1 inhibitors for the improvement of mainly systemic symptoms and using IL-6 inhibitors for the improvement of mainly chronic articular symptoms seem to be rational. Besides, due to both the potential adverse events and the high costs of the drugs, reducing the dose, lengthening the dosing interval, and ceasing the drugs should be the key points to be considered for patients with remission.

• #84 Study: Treat Adult-Onset Still’s Disease With Biologics | MedPage Today

  https://www.medpagetoday.com/rheumatology/arthritis/114954

  Study: Treat Adult-Onset Still’s Disease With Biologics […] Results from this cohort study showed that biologics were much more effective than conventional synthetic drugs as primary therapy. […] Patients with adult-onset Still’s disease were much more likely to achieve sustained remission when they were initially treated with biologic agents than corticosteroids or conventional disease-modifying anti-rheumatic drugs (DMARDs), a retrospective cohort study indicated. […] Half of patients receiving biologics as first-line therapy were in sustained event-free remission after 72 weeks, compared with 12% of those treated with conventional medications in the 86-person study. […] After statistical adjustments, odds of achieving sustained event-free remission — the study’s primary endpoint — were more than seven times greater for those receiving biologics.

• #85 Study: Treat Adult-Onset Still’s Disease With Biologics | MedPage Today

  https://www.medpagetoday.com/rheumatology/arthritis/114954

  „So far, the efficacy of first-line biological DMARD therapy versus conventional synthetic DMARD therapy for AOSD [adult-onset Still’s disease] has largely been extrapolated from studies in children,” Vordenbäumen and colleagues wrote. „Here, we provide evidence for more advantageous health benefits for first-line biological DMARD therapy versus conventional synthetic DMARD therapy in AOSD.” […] Biologics are indeed often used in the adult form, most commonly drugs that target the interleukin-1 (IL-1) pathway including anakinra (Kineret) and canakinumab (Ilaris) — unlike in other forms of inflammatory arthritis in which tumor necrosis factor inhibitors are usually the first biologic of choice. […] Yet registration trials of biologics for adult-onset Still’s disease have consistently missed their primary endpoints, Vordenbäumen’s group observed, although some have been approved in certain countries anyway. […] One of the worst complications of adult-onset Still’s disease is macrophage activation syndrome (MAS), which can be fatal. In fact, it was fatal for two patients in the conventional DMARD group. No one in the biologics group developed MAS nor did any die.

• #86 EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1614

  The TF thought it was important to highlight a few key points that should drive the management of patients with Stills disease. […] Since Stills disease evolution is often characterised by flares, it appeared relevant to the experts to apply the T2T principle for Stills disease management. […] The TF acknowledges the efficacy of GCs in managing Stills disease. […] Despite the small number of randomised controlled trials (RCTs) with IL-1 or IL-6 inhibitors performed in sJIA or AOSD, the TF strongly recommends their use based also on the overwhelming body of evidence from real-world experience supporting their efficacy to control all aspects of the disease including both systemic and joint manifestations and to limit exposure to GC. […] The TF recommends that the addition of the above-mentioned treatments is considered in patients with initial unsatisfactory response to high dose GCs and in patients with severe MAS and rapid worsening.

• #87 Clinical Challenges: Managing Adult-Onset Still’s Disease | MedPage Today

  https://www.medpagetoday.com/clinical-challenges/acr-adult-onset-stills-disease/102074

  While AOSD is treatable, it is not curable, noted Nilanjana Bose, MD, MBA, a rheumatologist at Lonestar Rheumatology in Houston. […] „I think it is a reasonably treatable disease if we get on the medications promptly,” said Morris, describing a patient with severe AOSD who responded to immunosuppressants and corticosteroids. […] Only one drug, the long-acting interleukin (IL)-1β inhibitor canakinumab (Ilaris) has FDA approval for use in the treatment of AOSD. […] While there are no guideline recommendations for the use of tocilizumab for the treatment of AOSD, an increasing number of reports have suggested that it is effective at treating AOSD. […] Likewise, a recent report found that while methotrexate and prednisolone were not enough for disease control in a case of elderly AOSD, combined use of methotrexate, tocilizumab, and prednisolone was effective to achieve remission. […] Rheumatologists continue to search for immunomodulatory agents that can help patients with AOSD who do not respond to canakinumab or tocilizumab.

• #88 EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1614

  Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Stills disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing. […] The TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Stills disease. […] Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). […] The recommendations emphasised the key role of expert centres for difficult-to-treat patients. […] These recommendations are the first consensus for the diagnosis and management of children and adults with Stills disease.

• #89 Get Adult-Onset Still’s Disease Care | Cleveland Clinic

  https://my.clevelandclinic.org/services/adult-onset-stills-disease-treatment

  Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen (Advil or Motrin) or aspirin may be the first course of action. We may also prescribe corticosteroids. Both kinds of medications help reduce fever, pain and inflammation. […] We offer advanced disease-modifying antirheumatic drugs (DMARDs) to manage severe and chronic adult-onset Stills disease. DMARDs work by suppressing your immune system. Your providers will figure out which medication can help protect your organs by controlling your immune system to reduce inflammation. […] Our physical therapists guide you through a personalized exercise routine designed to strengthen your joints. This will help relieve severe joint pain and prevent further damage and inflammation. Our occupational therapists help you learn ways to make everyday tasks easier and less painful to do.

• #90 FDA Approves First Treatment for Adult Onset Still’s Disease, a Severe and Rare Disease | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-adult-onset-stills-disease-severe-and-rare-disease

  Macrophage activation syndrome (MAS) is a known, life-threatening disorder that may develop in patients with rheumatic conditions, in particular Stills disease, and should be aggressively treated. Treatment with immunosuppressants may increase the risk of malignancies. Patients are advised not to receive live vaccinations during treatment.

• #91 Progress in biological therapies for adult-onset Stills | BTT

  https://www.dovepress.com/progress-in-biological-therapies-for-adult-onset-stills-disease-peer-reviewed-fulltext-article-BTT

  Adult-onset Stills disease (AOSD) is a rare multifactorial autoinflammatory disorder of unknown etiology, characterized by an excessive release of cytokines triggered by dysregulated inflammation and articular and systemic manifestations. […] Nowadays, the advances in biologic agents no longer limit the treatment to NSAIDs, glucocorticoids, or conventional synthetic DMARDs. The blockade of IL-1 and IL-6 is effective in the treatment of systemic and articular inflammation of AOSD patients; however, novel compounds with different properties and targets are now available and others are being studied. […] The treatment of AOSD should be addressed to the different variety and severity of symptoms manifested. Clearly, the control of systemic inflammation and the prevention of MAS remain the main therapeutical purposes. For all severe AOSD-related complications, high-dose corticosteroids and supportive measures remain the first-line treatment. In case of inadequate response, combination with IL1 or IL-6 blockers is adequate.

• #92 Successful Treatment of Recurrent Adult-Onset Still’s Disease wi | CCID

  https://www.dovepress.com/successful-treatment-of-recurrent-adult-onset-stills-disease-with-toci-peer-reviewed-fulltext-article-CCID

  As shown in this case, TCZ can rapidly control the symptoms of skin lesions, joint and muscle pain, and sore throat in adult patients with recurrent AOSD, effectively reduce all inflammatory indexes, and facilitate the reduction of glucocorticoid dose. […] However, the US Food and Drug Administration has not yet approved TCZ as a therapeutic drug for AOSD. […] Therefore, more multicenter collaborative research is needed as a basis to enhance the persuasiveness of TCZ as a treatment choice for AOSD patients, especially in low- and middle-income countries and regions. […] In addition, TCZ can also be used in refractory AOSD patients with macrophage activation syndrome. […] Consequently, extensive randomized controlled trials are essential in future work to further substantiate the efficacy and safety of TCZ in AOSD treatment, as well as to establish treatment guidelines.

• #93 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Stills-Disease.aspx

  Stills disease may lead to serious damage to the joints, specifically the knees, hips, and wrists. It can also impair lung and heart function. The goal of treatment for Stills disease or AOSD is to control the symptoms of arthritis and to reduce inflammation. […] The initial treatment is aimed at controlling the symptoms of joint inflammation and fever through anti-inflammatory drugs. The doctor may also prescribe corticosteroids to effectively control inflammation. […] The condition may take a toll on the joints. In severe cases of Stills disease, the patient may undergo joint replacement surgery. Patients with the condition should adhere to their medication regime to minimize inflammation and to prevent joint destruction.

• #94 adult Still’s disease « disease spotlights « JointHealth™ | changing arthritis

  https://jointhealth.org/aboutarthritis-diseasespotlight.cfm?id=1&locale=en-CA

  Corticosteroids, such as prednisone may be used to control high fever spikes, severe joint swelling and pain, and complications with internal organs. […] Methotrexate, a disease-modifying anti-rheumatic drug (DMARD), is often used in the treatment of adult Still’s disease. […] Biologic response modifiers, including anakinra (Kineret), infliximab (Remicade, Avsola, Inflectra, Renflexis, Remsima) and etanercept (Enbrel, Brenzys, Erelzi), have been used with success in a number of patients. […] The best way to avoid joint replacement is to develop a personalized plan with a rheumatologist. It is very important for people living with adult Still’s disease to keep to their medication regime and health promoting behaviours, as minimizing inflammation is key to preventing joint destruction. If joint damage progresses, surgery may be required. […] Learning to protect joints and pace activities can be helpful in managing adult Stills disease.

• #95 Get Adult-Onset Still’s Disease Care | Cleveland Clinic

  https://my.clevelandclinic.org/services/adult-onset-stills-disease-treatment

  Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen (Advil or Motrin) or aspirin may be the first course of action. We may also prescribe corticosteroids. Both kinds of medications help reduce fever, pain and inflammation. […] We offer advanced disease-modifying antirheumatic drugs (DMARDs) to manage severe and chronic adult-onset Stills disease. DMARDs work by suppressing your immune system. Your providers will figure out which medication can help protect your organs by controlling your immune system to reduce inflammation. […] Our physical therapists guide you through a personalized exercise routine designed to strengthen your joints. This will help relieve severe joint pain and prevent further damage and inflammation. Our occupational therapists help you learn ways to make everyday tasks easier and less painful to do.

• #96 adult Still’s disease « disease spotlights « JointHealth™ | changing arthritis

  https://jointhealth.org/aboutarthritis-diseasespotlight.cfm?id=1&locale=en-CA

  Corticosteroids, such as prednisone may be used to control high fever spikes, severe joint swelling and pain, and complications with internal organs. […] Methotrexate, a disease-modifying anti-rheumatic drug (DMARD), is often used in the treatment of adult Still’s disease. […] Biologic response modifiers, including anakinra (Kineret), infliximab (Remicade, Avsola, Inflectra, Renflexis, Remsima) and etanercept (Enbrel, Brenzys, Erelzi), have been used with success in a number of patients. […] The best way to avoid joint replacement is to develop a personalized plan with a rheumatologist. It is very important for people living with adult Still’s disease to keep to their medication regime and health promoting behaviours, as minimizing inflammation is key to preventing joint destruction. If joint damage progresses, surgery may be required. […] Learning to protect joints and pace activities can be helpful in managing adult Stills disease.

• #97 How to Cure Adult Still’s Disease?

  https://www.icliniq.com/articles/immune-disorders/adult-stills-disease

  […] […] What Lifestyle Changes Should People With Adult-Onset Stills Disease Implement? […] People under steroid treatment for adult Stills disease should check with their doctor and consume calcium and vitamin D supplements due to the risk of osteoporosis. […] Regular exercising is important. It might cause joint pain, but regular exercise improves joint flexibility and range of motion and is good for people with arthritis. […] Continue your medications regardless of the presence or absence of symptoms.

• #98 Adult Onset Still’s Disease Treatment In Singapore | Aaria Rheumatology

  https://aariarheumatology.com.sg/autoimmune-rheumatic-conditions/systemic-vasculitis-and-other-autoimmune-inflammatory-rheumatic-conditions/adult-onset-stills-disease/

  Regular Medical Follow-Up Monitor Progress Regular visits to your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns. […] Living with AOSD involves a combination of medical treatment, lifestyle changes, and self-care strategies. Here are some tips: Take medications as your healthcare provider prescribes to control inflammation and prevent disease progression. To maintain physical fitness and improve symptoms, engage in regular, low-impact exercises such as walking, swimming, or yoga. Eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health. Practice stress-reduction techniques such as meditation, deep breathing exercises, and mindfulness to help manage symptoms. Stay current with vaccinations and practice good hygiene to reduce the risk of infections, which can trigger flare-ups. Regularly visit your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns.

• #99 Health & Exercise in Adult-Onset Still’s DiseaseShare to Facebookprint pageBookmark for latercommentcaret iconcaret iconFollow us on facebookFollow us on instagramFollow us on facebookFollow us on linkedincaret icon

  https://raredisease.net/clinical/nutrition-exercise-adult-onset-stills-disease

  Adult-onset Still’s disease (AOSD) is a rare type of arthritis that can cause pain and joint damage. It is often managed with medicines that work on the immune system to reduce inflammation. These drugs can help prevent further joint damage and other related health problems. […] Maintaining a healthy weight helps your joints function smoothly and is an important part of a good AOSD treatment plan. […] A good exercise plan involves getting some form of moderate physical activity a few times a week. […] Before you start any new exercise plan, talk with your healthcare team about the best plan for you. […] However, not all supplements are good for you. […] Regular exercise and a balanced diet can also improve mood and reduce stress.

• #100 Adult onset Still disease

  https://dermnetnz.org/topics/adult-onset-still-disease

  There is no cure for adult-onset Still disease. The main goals of treatment are to reduce joint inflammation and avoid deformity. Therapy may include both medical and surgical interventions. In addition, the patient and their families need to fully understand the nature of the disease and how to live with it for the rest of their lives. […] Drug therapy to control many of the symptoms of Still disease includes: Aspirin, Nonsteroidal anti-inflammatory drugs such as ibuprofen, naproxen and indomethacin, Corticosteroids (e.g. prednisone), hydroxychloroquine, methotrexate, gold, azathioprine may be used for more severe symptoms, Biologics such as anakinra and antitumour necrosis factor therapies (adalimumab, etanercept, infliximab), and tocilizumab (an interleukin-6 receptor antagonist). […] Non-drug therapy includes physiotherapy, hydrotherapy and simply rest. Patients with Still disease will experience good and bad days. The number of bad days, that is flare-ups or exacerbations of the disease, can be controlled or reduced by careful planning of activities and rest periods. It has been shown that physical and emotional stress can exacerbate symptoms of Still disease.

• #101 Adult Onset Still’s Disease Treatment In Singapore | Aaria Rheumatology

  https://aariarheumatology.com.sg/autoimmune-rheumatic-conditions/systemic-vasculitis-and-other-autoimmune-inflammatory-rheumatic-conditions/adult-onset-stills-disease/

  Regular Medical Follow-Up Monitor Progress Regular visits to your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns. […] Living with AOSD involves a combination of medical treatment, lifestyle changes, and self-care strategies. Here are some tips: Take medications as your healthcare provider prescribes to control inflammation and prevent disease progression. To maintain physical fitness and improve symptoms, engage in regular, low-impact exercises such as walking, swimming, or yoga. Eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health. Practice stress-reduction techniques such as meditation, deep breathing exercises, and mindfulness to help manage symptoms. Stay current with vaccinations and practice good hygiene to reduce the risk of infections, which can trigger flare-ups. Regularly visit your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns.

• #102 Adult Onset Still’s Disease Treatment In Singapore | Aaria Rheumatology

  https://aariarheumatology.com.sg/autoimmune-rheumatic-conditions/systemic-vasculitis-and-other-autoimmune-inflammatory-rheumatic-conditions/adult-onset-stills-disease/

  Regular Medical Follow-Up Monitor Progress Regular visits to your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns. […] Living with AOSD involves a combination of medical treatment, lifestyle changes, and self-care strategies. Here are some tips: Take medications as your healthcare provider prescribes to control inflammation and prevent disease progression. To maintain physical fitness and improve symptoms, engage in regular, low-impact exercises such as walking, swimming, or yoga. Eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health. Practice stress-reduction techniques such as meditation, deep breathing exercises, and mindfulness to help manage symptoms. Stay current with vaccinations and practice good hygiene to reduce the risk of infections, which can trigger flare-ups. Regularly visit your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns.

• #103 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Testing prior to treatment initiation: Prior to initiation of any therapy, a complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) levels, serum creatinine levels, and liver profile should be ordered. The treatment options provided below are listed in the order of management for increasing disease severity. Expert consultation is recommended for guidance regarding treatment decision making. […] Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs are often used during the diagnostic phase of AOSD, but their clinical usefulness is limited. Retrospective cohort studies have shown NSAIDs to be ineffective in controlling symptoms of AOSD, with 80% of patients failing to respond. In addition, up to 20% of patients experienced an adverse event. Therefore, NSAIDs are not an effective agent in controlling disease activity. They may be used as adjunctive therapy for arthritic symptoms, but high-quality evidence for their use in this role is lacking. NSAIDs could be tried alone in a mild form of the disease for a limited period.

• #104 Adult Still disease | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/adult-still-disease

  Adult Still disease can damage joints, particularly the wrists. Treatment involves medicine to reduce pain and help control the disease. Prednisone is often used if pain relievers such as ibuprofen (Advil, Motrin IB, others) are not enough. […] A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects. […] Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function. […] Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes.

• #105 Adult-Onset Still Disease – What You Need to Know

  https://www.drugs.com/cg/adult-onset-still-disease.html

  Disease-modifying antirheumatic drugs (DMARDs) help slow joint damage, and relieve pain and inflammation. DMARDs also help protect your joint function. […] The following list of medications are related to or used in the treatment of this condition: tocilizumab, anakinra, Ilaris, canakinumab, etanercept. […] Keep taking your medicine, even if you feel better. AOSD flares come and go. In between flares, you may feel well. It is important to keep taking your medicine as directed. DMARDs need to be taken to help keep AOSD from getting worse. Steroids help reduce inflammation. Do not stop taking steroids suddenly. A sudden stop can be dangerous. Your provider will help you take less medicine over time until you can stop safely.

• #106 Adult-Onset Still Disease – What You Need to Know

  https://www.drugs.com/cg/adult-onset-still-disease.html

  Disease-modifying antirheumatic drugs (DMARDs) help slow joint damage, and relieve pain and inflammation. DMARDs also help protect your joint function. […] The following list of medications are related to or used in the treatment of this condition: tocilizumab, anakinra, Ilaris, canakinumab, etanercept. […] Keep taking your medicine, even if you feel better. AOSD flares come and go. In between flares, you may feel well. It is important to keep taking your medicine as directed. DMARDs need to be taken to help keep AOSD from getting worse. Steroids help reduce inflammation. Do not stop taking steroids suddenly. A sudden stop can be dangerous. Your provider will help you take less medicine over time until you can stop safely.

• #107 Adult-onset Still’s Disease Treatment Market | AoSD Treatment Market

  https://www.delveinsight.com/blog/adult-onset-stills-disease-treatment-market

  Adult-onset Stills Disease (AoSD) is a multigenic autoinflammatory disorder that stands at the junction of autoinflammatory and autoimmune diseases and involves innate and adaptive immune systems. The treatment of AoSD is currently centred on nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, and antirheumatic agents. According to older studies, NSAID as monotherapy effectively controls disease in only 715% of patients, and most patients are treated with steroids at some point in their disease course, with responses ranging from 76% to 95%, making corticosteroids the cornerstone of AoSD therapy. […] Many different therapies have been investigated in Adult-onset Stills Disease treatment market, but none has proven effective consistently. Therefore, a variety of monotherapies and combinational therapies are used to treat the affected population. The first-line therapy of AoSD consists of corticosteroid and NSAID, while the second-line therapy includes methotrexate (MTX) or cyclosporin A. In the case of refractory AoSD, the preferred option is the usage of IL-1 or IL-6 antagonists along with more biologics varying among individuals.

• #108 Adult-onset Still’s Disease Treatment Market | AoSD Treatment Market

  https://www.delveinsight.com/blog/adult-onset-stills-disease-treatment-market

  Adult-onset Stills Disease (AoSD) is a multigenic autoinflammatory disorder that stands at the junction of autoinflammatory and autoimmune diseases and involves innate and adaptive immune systems. The treatment of AoSD is currently centred on nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, and antirheumatic agents. According to older studies, NSAID as monotherapy effectively controls disease in only 715% of patients, and most patients are treated with steroids at some point in their disease course, with responses ranging from 76% to 95%, making corticosteroids the cornerstone of AoSD therapy. […] Many different therapies have been investigated in Adult-onset Stills Disease treatment market, but none has proven effective consistently. Therefore, a variety of monotherapies and combinational therapies are used to treat the affected population. The first-line therapy of AoSD consists of corticosteroid and NSAID, while the second-line therapy includes methotrexate (MTX) or cyclosporin A. In the case of refractory AoSD, the preferred option is the usage of IL-1 or IL-6 antagonists along with more biologics varying among individuals.

• #109 Treatment of adult-onset Still’s disease: a review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278737/

  Adult-onset Stills disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. […] The first part of this review deals with the treatments that are currently available for AOSD. […] Given that the current information on treatment efficacy is obtained from small retrospective case series and not from prospective, double-blind, randomized trials, the treatment of AOSD remains empirical. […] Corticosteroids remain the first-line treatment for AOSD, regardless of the clinical presentation. […] In the event of failure of corticosteroid treatment or steroid-dependence, disease-modifying anti-rheumatic drugs (DMARDS) can be considered. […] Methotrexate has proved beneficial and remains the first-line steroid-sparing treatment in AOSD.

• #110 Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-2021-9

  Adult-onset Stills disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. There was a large consensus that failure of the first IL-1 inhibitor does not preclude response to another one. IL-1 inhibition represents an effective therapeutic approach in AOSD refractory to conventional treatment and/or other biologics. In AOSD, treatment with IL-1 Inhibitors is effective on different clinical and laboratory parameters and displays a significant steroid-sparing effect in most patients. IL-1 inhibitors are effective in the treatment of AOSD-related Macrophage Activation Syndrome (MAS). IL-1 Inhibitors have an overall satisfactory safety profile in AOSD. Based on our review of the literature, therapies targeting IL-1 (anakinra, canakinumab, and rilonacept) are significantly effective in patients with AOSD refractory to conventional treatment. The effectiveness of anti-IL-1 therapies in AOSD ranged from 50 to 100%. Data on the efficacy of anti-IL1 agents in the treatment of arthritis in patients with AOSD are still controversial. A different profile of efficacy among IL-1 inhibitors cannot be established, because head-to-head comparisons are lacking. Indirect data show that IL-1 inhibitors can be effective in AOSD, both in first and subsequent lines of biologic treatment. Some data suggest that IL-1 inhibitors may be more effective on systemic rather than chronic articular manifestations of AOSD.

• #111 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  Objectives To analyse the efficacy and safety of treatments for Stills disease and macrophage activation syndrome (MAS). […] IL-1i and IL-6Ri show the highest level of efficacy in the treatment of Stills disease. For MAS, IL-1 and interferon- inhibition appear to be effective on a background of high-dose glucocorticoids. […] Interleukin-1 inhibitors (IL-1i) and IL-6Ri show the highest level of evidence in terms of efficacy, safety and an acceptable risk-benefit ratio for the treatment of sJIA and AOSD. […] IL-1 and IL-6R inhibitors show the most interesting risk-benefit ratio compared with all alternatives in Stills disease. In MAS, despite the scarcity of data, high-dose glucocorticoids combined with IL-1 or IFN- inhibition appear presently to be the best available strategy. […] The therapeutic approach in both sJIA and AOSD historically relies on non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids (GCs).

• #112 Efficacy and safety of therapies for Still’s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still’s disease | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/12/1731

  Objectives To analyse the efficacy and safety of treatments for Stills disease and macrophage activation syndrome (MAS). […] IL-1i and IL-6Ri show the highest level of efficacy in the treatment of Stills disease. For MAS, IL-1 and interferon- inhibition appear to be effective on a background of high-dose glucocorticoids. […] Interleukin-1 inhibitors (IL-1i) and IL-6Ri show the highest level of evidence in terms of efficacy, safety and an acceptable risk-benefit ratio for the treatment of sJIA and AOSD. […] IL-1 and IL-6R inhibitors show the most interesting risk-benefit ratio compared with all alternatives in Stills disease. In MAS, despite the scarcity of data, high-dose glucocorticoids combined with IL-1 or IFN- inhibition appear presently to be the best available strategy. […] The therapeutic approach in both sJIA and AOSD historically relies on non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids (GCs).

• #113 Study: Treat Adult-Onset Still’s Disease With Biologics | MedPage Today

  https://www.medpagetoday.com/rheumatology/arthritis/114954

  Study: Treat Adult-Onset Still’s Disease With Biologics […] Results from this cohort study showed that biologics were much more effective than conventional synthetic drugs as primary therapy. […] Patients with adult-onset Still’s disease were much more likely to achieve sustained remission when they were initially treated with biologic agents than corticosteroids or conventional disease-modifying anti-rheumatic drugs (DMARDs), a retrospective cohort study indicated. […] Half of patients receiving biologics as first-line therapy were in sustained event-free remission after 72 weeks, compared with 12% of those treated with conventional medications in the 86-person study. […] After statistical adjustments, odds of achieving sustained event-free remission — the study’s primary endpoint — were more than seven times greater for those receiving biologics.

• #114 Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs | The Journal of Rheumatology

  https://www.jrheum.org/content/51/5/442

  In conclusion, evidence from case series appears to support the case for bDMARDs for AOSD, particularly TCZ, ANK, and CNK. However, the magnitude of effect and comparative effectiveness of different treatments is still uncertain. Standardization of outcomes and outcome definitions should drive future research protocols, with studies of adequate duration. To reduce bias in studies comparing interventions, treatments should be given at similar stages in the treatment pathway.

• #115 Study: Treat Adult-Onset Still’s Disease With Biologics | MedPage Today

  https://www.medpagetoday.com/rheumatology/arthritis/114954

  Study: Treat Adult-Onset Still’s Disease With Biologics […] Results from this cohort study showed that biologics were much more effective than conventional synthetic drugs as primary therapy. […] Patients with adult-onset Still’s disease were much more likely to achieve sustained remission when they were initially treated with biologic agents than corticosteroids or conventional disease-modifying anti-rheumatic drugs (DMARDs), a retrospective cohort study indicated. […] Half of patients receiving biologics as first-line therapy were in sustained event-free remission after 72 weeks, compared with 12% of those treated with conventional medications in the 86-person study. […] After statistical adjustments, odds of achieving sustained event-free remission — the study’s primary endpoint — were more than seven times greater for those receiving biologics.

• #116 Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11147545/

  Adult-onset Stills disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis; most doctors treating AOSD in the Netherlands treat 5 patients per year. Currently, there is no internationally accepted treatment guideline for AOSD. […] The panel consented on reduction of the use of glucocorticoids to avoid side effects, and preferred the use of biologics over conventional treatment. The role of IL-1 and IL-6 blocking agents was considered important in the treatment of AOSD. […] Anti-inflammatory drugs form the cornerstone of treatment of AOSD. Treatment is often based on expert opinion through the lack of international guidelines. Historically, glucocorticoids (GC) are considered the backbone of treatment. MTX is often the first conventional DMARD used to spare GC. More recently, IL-1 and IL-6 were identified as important mediators of inflammation in AOSD. Biologics targeting these pathways are approved by both the FDA and the EMA for the treatment of sJIA and AOSD.

• #117 Adult Onset Still’s Disease Treatment In Singapore | Aaria Rheumatology

  https://aariarheumatology.com.sg/autoimmune-rheumatic-conditions/systemic-vasculitis-and-other-autoimmune-inflammatory-rheumatic-conditions/adult-onset-stills-disease/

  Adult Onset Stills Disease is a complex and serious condition that requires comprehensive management. While there is currently no cure, with early diagnosis and appropriate treatment, many individuals can manage their symptoms and maintain a good quality of life. If you suspect you have AOSD or are experiencing symptoms suggestive of the condition, its essential to seek medical evaluation and treatment promptly. By working closely with your healthcare team, adhering to treatment plans, and making healthy lifestyle choices, you can effectively manage AOSD and improve your quality of life.

• #118 Adult Onset Still’s Disease | SMC Physicians, New Jersey

  https://smc-physicians.com/service/adult-onset-stills-disease-nj/

  Adult Onset Stills Disease typically begins in adulthood. […] Many people with AOSD can live a full and normal life with the right treatment. […] How is AOSD Treated? […] Non-Steroidal Anti-Inflammatory Drugs (NSAIDs): NSAIDs can help reduce the pain, inflammation, and fever associated with AOSD. […] Corticosteroids: In more severe cases, corticosteroids may be prescribed to suppress the overactive immune response. […] Disease-Modifying Antirheumatic Drugs (DMARDs): DMARDs, such as methotrexate or biologics, are often used to control symptoms and prevent long-term joint damage. […] Supportive Care: Rest, physical therapy, and lifestyle modifications can play a significant role in managing symptoms and improving overall well-being. […] With early diagnosis and appropriate treatment, many patients can effectively manage symptoms and lead fulfilling lives.

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