Materiały źródłowe

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

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

  Adult-onset Still disease (AOSD) is a disease occurring in persons aged 16 years and resembling a systemic form of juvenile idiopathic arthritis. It may be associated with fever, rash, lymphadenopathy, splenomegaly, serositis, and inflammation of multiple organs. AOSD develops after the age of 16. In adults the symptoms may be due to the first occurrence of the disease or a relapse. AOSD may follow a self-limiting (lasting 1 year), relapsing (relapses may be very frequent), or chronic (symptoms are continuously present throughout the year) course. In some patients significant joint destruction occurs. […] Symptoms (Table 1): […] 1) Most frequent: […] a) Fever (80% of cases; usually 39 degrees Celsius, most commonly in the evening or twice during a 24-h period). […] b) Sore throat with features of inflammation (50%; frequently develops several days or weeks prior to other symptoms).

• #2 Azthena logo with the word Azthena

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

  Stills disease is a rare inflammatory condition characterized by arthritis along with recurrent orange-to-pink colored skin rashes and high spiking fevers. […] In adults, it is referred to as adult-onset Stills disease (AOSD). AOSD has relatively less prevalence (1 in 100,000 adults) compared to SOJIA. Also, AOSD is found to be more common in females. […] The majority of patients with Stills disease suffer from high spiking fever of approximately 39C or even higher, which returns to normal temperature within a few hours. The fever occurs recurrently during the same time of the day (commonly in the afternoon or evening). Some patients experience multiple fever episodes within a single day. […] Salmon-to-pink colored skin rashes are also commonly seen in patients with Stills disease. Rashes mainly appear on the chest region and thighs, and less commonly on upper and lower limbs. Appearance of rashes is also usually in sync with the fever spikes. The extent of itchiness of the rashes varies amongst the patients.

• #3 Still’s disease vs. rheumatoid arthritis: What to know

  https://www.medicalnewstoday.com/articles/stills-disease-vs-rheumatoid-arthritis

  Stills disease causes inflammation that can affect the entire body, resulting in: joint pain, joint swelling, skin rashes. […] The primary clinical symptoms of Stills disease are: fever, salmon pink rash on lighter skin or evanescent rash on dark skin tones, joint pain, joint stiffness. These symptoms occur in 75-95% of people with Stills disease. Other common symptoms of Stills disease include: muscle aches and pains, or myalgia, sore throat, swollen lymph nodes, an enlarged spleen. Less common symptoms of Stills disease include: an enlarged liver, pleurisy, or inflammation of the outer lining of the lungs, which causes chest pain, pericarditis, or inflammation of the sac that surrounds the heart, abdominal pain. […] Stills disease causes inflammation in the body. Common symptoms include fever, rash, and joint pain.

• #4 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  A rare inflammatory multisystem disorder characterized clinically by four cardinal signs: fever of unknown origin, arthralgia or arthritis, hyperleucocytosis, and typical skin rash. […] The estimated prevalence of AOSD is more than 1/100,000 population. There is a slightly higher number of affected women than affected men. […] AOSD primarily affects young adults, although older patients have been reported. The presenting features are variable and may include high fever (39C) with daily spikes, sore throat or pharyngitis, arthralgia or arthritis (65% of patients), transient maculopapular rash, and more rarely myalgia, lymphadenopathy, hepatosplenomegaly, and serositis. […] Three different disease courses have been described: self-limited, systemic course with a single flare and complete remission within 2 to 4 weeks, intermittent course with recurrence of systemic or articular flares after remission of 2 weeks to 2 years, and a primarily articular chronic course (with erosions in a 1/3 patients).

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

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

  The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […] Around one-third of people diagnosed with the condition will have it for less than a year before their symptoms ease. They might need to stay on drug treatment for a bit longer to make sure the condition is completely under control. Another third of people will have flares of the condition before symptoms disappear completely. The flares can sometimes be years apart, and they don’t always affect the joints. Symptoms may get easier to manage in later flares.

• #6 Adult Still disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/symptoms-causes/syc-20351907

  Adult Still disease is a rare type of inflammatory arthritis. Common symptoms are fevers, rash and joint pain. The condition can occur in some people as a single episode that goes away. In other people, the condition doesn’t go away, or it goes away but comes back. […] Most people with adult Still disease have a combination of the following symptoms: […] Fever may rise to at least 102 degrees Fahrenheit (38.9 degrees Celsius). The fever may spike once or twice a day for a week or longer. […] A rash might come and go with the fever. The rash usually appears on the trunk, arms or legs. […] This is one of the first symptoms of adult Still disease. The lymph nodes in the neck might be swollen and tender. […] Joints especially in the knees and wrists might be stiff, painful and inflamed. Ankles, elbows, hands and shoulders also might ache. The joint discomfort usually lasts at least two weeks.

• #7 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. It can cause a variety of systemic symptoms, most often muscle pain, a fever and a rash. Sometimes, its temporary, and sometimes, its long-lasting. Anti-inflammatory medications can help suppress the inflammation. […] Adult-onset Stills disease is a form of inflammatory arthritis. In addition to joint pain, it causes a daily high fever that comes with a rash. […] Adult-onset Stills disease (AOSD) is a rare type of inflammatory arthritis that affects your whole body. It causes joint and muscle pain, a daily fever and a rash. It can also cause other systemic (whole-body) symptoms. […] AOSD is an adult form of juvenile Stills disease (juvenile idiopathic arthritis, JIA). It typically occurs in younger adults, between the ages of 16 and 35. Like JIA, the condition may be temporary, lasting less than a year, or may come and go for a long time. Symptom flares may occur regularly or far apart, and they may be mild to severe. Medications can help manage your symptoms when they arise.

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

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

  Adult-onset Stills disease (AOSD) refers to a rare inflammatory condition that affects multiple organs and usually presents in young adults. The symptoms typically include fever, rash, and joint pain. […] AOSD is a rare auto-inflammatory disease that affects the entire body. The cause of the condition is unknown, but it results in characteristic symptoms that include fever, a pink-colored rash, and joint pain. AOSD presents in adulthood, with most cases beginning between 16 and 35 years of age. […] The symptoms, course, and severity of AOSD vary from person to person. The condition may appear suddenly and disappear. A person may develop and experience a single episode and then have no symptoms for several years. In other people, the condition persists. The three hallmark symptoms present in 75-95% of those affected are: Fever: A person may experience a fever higher than 39C that recurs daily and returns to normal between spikes. It often occurs later in the day and precedes other symptoms. Rashes: A visible symptom of AOSD is a pink or salmon-colored maculopapular rash that is not itchy. The rash predominantly appears on the trunk and parts of the limbs near the trunk. Heat or rubbing the skin can induce the rash. Joint pain: Initially, joint pain may be mild and transient, affecting fewer than five joints. Commonly involved joints include the knees, wrists, and ankles. However, it may progress and become severe and destructive, affecting multiple joints, including small ones.

• #9 Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-023-02651-3

  Adult-onset Stills disease (AOSD) is a multi-system, auto-inflammatory disease characterized by fever, arthralgia, typical rash, leukocytosis, sore throat, and liver dysfunction, among other symptoms. […] The lab values for inflammatory markers were increased. Co-diagnoses such as rash, sore throat, and fever appeared in all AOSD cohorts, with the highest occurrences in the AOSD+COVID-19 vaccination+SARS-CoV-2 infection cohort. […] The incidence of AOSD doubled for the Cov cohort and Vac cohort. Moreover, the incidence of AOSD was 4.82 times higher for Vac+Cov cohort. […] The two-fold higher incidence of AOSD in Vac cohort compared with the primary cohort supports the assumption of a connection between AOSD and COVID-19 vaccination. […] The Vac+Cov cohort has the highest incidence of AOSD. […] Even though the incidence of AOSD increased significantly after SARS-CoV-2 infection and/or COVID-19 vaccination, AOSD remains an orphan disease.

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

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

  Adult-onset Still disease (AOSD) is a disease occurring in persons aged 16 years and resembling a systemic form of juvenile idiopathic arthritis. It may be associated with fever, rash, lymphadenopathy, splenomegaly, serositis, and inflammation of multiple organs. AOSD develops after the age of 16. In adults the symptoms may be due to the first occurrence of the disease or a relapse. AOSD may follow a self-limiting (lasting 1 year), relapsing (relapses may be very frequent), or chronic (symptoms are continuously present throughout the year) course. In some patients significant joint destruction occurs. […] Symptoms (Table 1): […] 1) Most frequent: […] a) Fever (80% of cases; usually 39 degrees Celsius, most commonly in the evening or twice during a 24-h period). […] b) Sore throat with features of inflammation (50%; frequently develops several days or weeks prior to other symptoms).

• #11 Clinical features and prognosis of adult-onset Still’s disease: 75 cases from China

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4659083/

  This study evaluated the clinical characteristics, treatment outcomes, and complications of patients with adult onset Stills disease (AOSD) in our local Chinese population. The most common presenting features were fever (96%), arthritis (57.33%), rash (78.67%), and sore throat (49.3%). Patients with AOSD had complex symptoms with no specific laboratory findings. The clinical presentation of AOSD is nonspecific and heterogeneous. Symptoms commonly include high spiking fever, arthralgia or arthritis, transient maculopapular rash, and sore throat. The prognosis of AOSD is typically good, but the relapse rate is high. The cumulative relapse rate was 45.3%. Most patients (92%) required corticosteroid therapy; of these, 33.3% also received disease-modifying antirheumatic drugs or immunosuppressive drugs. Sixty-four and 45.33% patients with AOSD achieved partial and complete remission, respectively, after 2 weeks of treatment, and 92% and 74.67%, respectively, after 1 month. Elevated serum transaminase levels were transient and returned to normal after corticosteroid or NSAIDs treatment in most patients. However, serum transaminase levels were higher during treatment in 5 patients, and became high in 2 patients whose serum transaminase levels were initially normal.

• #12 Adult onset Still disease

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

  Intermittent high fever occurs in all patients. Temperatures may get up to 39 or 40C before rapidly returning to normal levels. These fever spikes often occur at nearly the same time every day and may be accompanied by a feeling of extreme fatigue. […] Skin rash develops in about of 95% of patients. Characteristics of the rash are: Rash comes and goes with the fever, Small, non-itchy spots or patches up to 5cm in diameter, Salmon-pink coloured, Usually appear on the limbs and trunk, but may occur on the face or neck. […] Other systemic symptoms include: Swollen lymph glands and/or enlarged spleen/liver in 85% of patients, Muscle pain coinciding with fever, Inflamed lungs (pleuritis) and/or surrounding heart (pericarditis) in 60% of patients, Marked increase in white blood cell count, Severe anaemia, Abdominal pain.

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

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

  The primary symptoms include: Fever. When symptoms are active, most people have a daily fever with two spikes, one in the morning and one in the evening. Fever spikes are typically over 102 degrees Fahrenheit (39 degrees Celsius). Skin rash. A pink or salmon-colored rash usually appears when your fever is spiking. It may disappear in between spikes. It usually affects your chest, arms and legs first, but may spread. Joint pain. Most people feel achy in their joints, and many also experience swelling and stiffness. It may start in a few joints at first, but after a while, you may start to feel it all over. Muscle pain. You may also feel achy in your muscles, especially when your fever is spiking. […] In most cases, AOSD will impact your quality of life more than your long-term health. Its uncomfortable and can interfere with day-to-day activities, but it wont necessarily do lasting damage to your body.

• #14 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  Adult-onset Still’s disease (AOSD) is an uncommon systemic inflammatory disorder characterized by inflammatory polyarthritis, daily fever, and a transient salmon-pink maculopapular rash. A distinguishing feature is a frequently elevated serum ferritin level exceeding 1000 ng/ml. […] The key clinical features observed in AOSD include fever, rash, and arthritis or arthralgia, occurring in approximately 75% to 95% of patients. Other common symptoms encompass myalgia, pharyngitis, lymphadenopathy, and splenomegaly. Less frequently observed symptoms comprise hepatomegaly, pleurisy, pericarditis, and abdominal pain. […] Fever in AOSD usually follows a quotidian pattern (daily recurring fever with temperature returning to normal between fever spikes), often occurring late during the day and generally preceding other symptoms.

• #15 Adult Still Disease – UF Health

  https://ufhealth.org/conditions-and-treatments/adult-still-disease

  Adult Still disease (ASD) is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis. […] Almost all people with the disease will have fever, joint pain, sore throat, and a rash. […] Joint pain, warmth, and swelling are common. Most often, several joints are involved at the same time. Often, people with the condition have morning stiffness of joints that lasts for at least one hour. […] The fever comes on quickly once per day, most commonly in the afternoon or evening. […] The skin rash is often salmon-pink colored and comes and goes with the fever. […] Symptoms continue for a long time (chronic) in about one third of people with adult Still disease.

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

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

  c) Salmon-pink macular or maculopapular rash (80%; often transient, develops only during episodes of fever, rarely pruritic, most frequently located on the trunk and proximal limbs, less often on the face; it may be triggered by heat [eg, following a hot bath] or skin abrasion [eg, scratchy clothing]). […] d) Arthralgia (90%; worsens during febrile periods; occasionally arthritis, most commonly affecting the knees and wrists; approximately one-fourth of patients develop ankyloses of the involved joints). […] e) Myalgia (80%). […] f) Lymphadenopathy (50%; most frequently affecting the cervical lymph nodes; the lymph nodes may be tender; retroperitoneal lymphadenopathy may cause abdominal pain that is difficult to diagnose). […] g) Splenomegaly, hepatomegaly (50%). […] 2) Less frequent: Symptoms of pleuritis or pericarditis, weight loss.

• #17 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Stills Disease is a rare inflammatory disease that exists in two main forms: adult-onset Stills disease (AOSD), and a type of childhood arthritis called systemic juvenile idiopathic arthritis (SJIA). Symptoms include fatigue, fever, joint pain, and inflammation. […] Both the adult form and the juvenile form of Stills disease are marked by fatigue, fever, joint pain, and inflammation. A distinctive salmon-coloured rash is often present, and several of the body’s organs can be affected. […] The main symptoms of the condition include fatigue, muscle pain, sore throat, high fever reaching up to 41C, a salmon-pink rash, and inflammatory arthritis which tends to be more severe in the juvenile form. […] For the juvenile form of the disease, up to 30% of children with SJIA still experience symptoms 10 years after their first symptoms. Symptoms can persist into adulthood. AOSD generally follows one of three main patterns: Monophasic pattern: a single episode of symptoms that typically lasts weeks to months, but usually less than a year. Polyphasic (intermittent) pattern: more than one episode of symptoms. Patients usually remain free from symptoms for weeks to years between episodes. Chronic pattern: patients with chronic AOSD have persistent symptoms over time.

• #18 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  The rash in AOSD is classically characterized as evanescent, displaying a salmon-colored, macular, or maculopapular appearance. […] Arthritis in AOSD may initially present as mild, transient, and oligoarticular, potentially progressing into severe, destructive, and symmetric polyarticular forms. […] Myalgia tends to exacerbate during fever spikes and can occasionally be severe and debilitating. […] Symmetrical slightly tender lymphadenopathy is reported in one-third to two-thirds of patients with AOSD, while splenomegaly is observed in one-third to one-half of patients. […] The course of AOSD generally aligns with 1 of 3 patterns: self-limited illness, intermittent flares, or chronic Still disease. Predictors for chronicity and unfavorable outcomes include the presence of erosive polyarthritis at the time of presentation and involvement of shoulders or hips.

• #19 Adult onset Still disease

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

  Intermittent high fever occurs in all patients. Temperatures may get up to 39 or 40C before rapidly returning to normal levels. These fever spikes often occur at nearly the same time every day and may be accompanied by a feeling of extreme fatigue. […] Skin rash develops in about of 95% of patients. Characteristics of the rash are: Rash comes and goes with the fever, Small, non-itchy spots or patches up to 5cm in diameter, Salmon-pink coloured, Usually appear on the limbs and trunk, but may occur on the face or neck. […] Other systemic symptoms include: Swollen lymph glands and/or enlarged spleen/liver in 85% of patients, Muscle pain coinciding with fever, Inflamed lungs (pleuritis) and/or surrounding heart (pericarditis) in 60% of patients, Marked increase in white blood cell count, Severe anaemia, Abdominal pain.

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

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

  c) Salmon-pink macular or maculopapular rash (80%; often transient, develops only during episodes of fever, rarely pruritic, most frequently located on the trunk and proximal limbs, less often on the face; it may be triggered by heat [eg, following a hot bath] or skin abrasion [eg, scratchy clothing]). […] d) Arthralgia (90%; worsens during febrile periods; occasionally arthritis, most commonly affecting the knees and wrists; approximately one-fourth of patients develop ankyloses of the involved joints). […] e) Myalgia (80%). […] f) Lymphadenopathy (50%; most frequently affecting the cervical lymph nodes; the lymph nodes may be tender; retroperitoneal lymphadenopathy may cause abdominal pain that is difficult to diagnose). […] g) Splenomegaly, hepatomegaly (50%). […] 2) Less frequent: Symptoms of pleuritis or pericarditis, weight loss.

• #21 Adult Still disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/symptoms-causes/syc-20351907

  Adult Still disease is a rare type of inflammatory arthritis. Common symptoms are fevers, rash and joint pain. The condition can occur in some people as a single episode that goes away. In other people, the condition doesn’t go away, or it goes away but comes back. […] Most people with adult Still disease have a combination of the following symptoms: […] Fever may rise to at least 102 degrees Fahrenheit (38.9 degrees Celsius). The fever may spike once or twice a day for a week or longer. […] A rash might come and go with the fever. The rash usually appears on the trunk, arms or legs. […] This is one of the first symptoms of adult Still disease. The lymph nodes in the neck might be swollen and tender. […] Joints especially in the knees and wrists might be stiff, painful and inflamed. Ankles, elbows, hands and shoulders also might ache. The joint discomfort usually lasts at least two weeks.

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

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

  c) Salmon-pink macular or maculopapular rash (80%; often transient, develops only during episodes of fever, rarely pruritic, most frequently located on the trunk and proximal limbs, less often on the face; it may be triggered by heat [eg, following a hot bath] or skin abrasion [eg, scratchy clothing]). […] d) Arthralgia (90%; worsens during febrile periods; occasionally arthritis, most commonly affecting the knees and wrists; approximately one-fourth of patients develop ankyloses of the involved joints). […] e) Myalgia (80%). […] f) Lymphadenopathy (50%; most frequently affecting the cervical lymph nodes; the lymph nodes may be tender; retroperitoneal lymphadenopathy may cause abdominal pain that is difficult to diagnose). […] g) Splenomegaly, hepatomegaly (50%). […] 2) Less frequent: Symptoms of pleuritis or pericarditis, weight loss.

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

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

  c) Salmon-pink macular or maculopapular rash (80%; often transient, develops only during episodes of fever, rarely pruritic, most frequently located on the trunk and proximal limbs, less often on the face; it may be triggered by heat [eg, following a hot bath] or skin abrasion [eg, scratchy clothing]). […] d) Arthralgia (90%; worsens during febrile periods; occasionally arthritis, most commonly affecting the knees and wrists; approximately one-fourth of patients develop ankyloses of the involved joints). […] e) Myalgia (80%). […] f) Lymphadenopathy (50%; most frequently affecting the cervical lymph nodes; the lymph nodes may be tender; retroperitoneal lymphadenopathy may cause abdominal pain that is difficult to diagnose). […] g) Splenomegaly, hepatomegaly (50%). […] 2) Less frequent: Symptoms of pleuritis or pericarditis, weight loss.

• #24 Clinical features and prognosis of adult-onset Still’s disease: 75 cases from China

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4659083/

  This study evaluated the clinical characteristics, treatment outcomes, and complications of patients with adult onset Stills disease (AOSD) in our local Chinese population. The most common presenting features were fever (96%), arthritis (57.33%), rash (78.67%), and sore throat (49.3%). Patients with AOSD had complex symptoms with no specific laboratory findings. The clinical presentation of AOSD is nonspecific and heterogeneous. Symptoms commonly include high spiking fever, arthralgia or arthritis, transient maculopapular rash, and sore throat. The prognosis of AOSD is typically good, but the relapse rate is high. The cumulative relapse rate was 45.3%. Most patients (92%) required corticosteroid therapy; of these, 33.3% also received disease-modifying antirheumatic drugs or immunosuppressive drugs. Sixty-four and 45.33% patients with AOSD achieved partial and complete remission, respectively, after 2 weeks of treatment, and 92% and 74.67%, respectively, after 1 month. Elevated serum transaminase levels were transient and returned to normal after corticosteroid or NSAIDs treatment in most patients. However, serum transaminase levels were higher during treatment in 5 patients, and became high in 2 patients whose serum transaminase levels were initially normal.

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

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

  Adult-onset Stills disease is an autoimmune condition. This means that the condition is caused by your body’s immune system. AOSD can cause pain and stiffness in your joints, as well as inflammation in other areas of your body. […] People who develop AOSD often have the following symptoms: a fever, a sore throat, arthritis or joint pain, a salmon-pink rash that comes and goes quickly. Some people may not have all these symptoms at once, which can make it difficult to diagnose the condition. The fever caused by AOSD usually goes up and down a few times during the day, usually going up in the afternoon or evening. But some people may have a fever all the time or have spikes in the fever in the mornings. […] People can also have pain in their muscles or joints, or unexpected weight loss before the fevers start. AOSD can also cause fatigue, which is an overwhelming feeling of tiredness that doesn’t always get better with sleep or rest. People with AOSD frequently experience joint pain, going on to develop arthritis. Arthritis causes pain, swelling and stiffness in the joints. AOSD most commonly affects the knees, wrists and ankles, but it can also affect the hands, feet, hips, elbows, shoulders and jaw.

• #26 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  The rash in AOSD is classically characterized as evanescent, displaying a salmon-colored, macular, or maculopapular appearance. […] Arthritis in AOSD may initially present as mild, transient, and oligoarticular, potentially progressing into severe, destructive, and symmetric polyarticular forms. […] Myalgia tends to exacerbate during fever spikes and can occasionally be severe and debilitating. […] Symmetrical slightly tender lymphadenopathy is reported in one-third to two-thirds of patients with AOSD, while splenomegaly is observed in one-third to one-half of patients. […] The course of AOSD generally aligns with 1 of 3 patterns: self-limited illness, intermittent flares, or chronic Still disease. Predictors for chronicity and unfavorable outcomes include the presence of erosive polyarthritis at the time of presentation and involvement of shoulders or hips.

• #27 Adult Still’s Disease

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

  This rare type of inflammatory arthritis is marked by fever, rash and joint pain. […] Adult-onset Stills disease is a rare type of arthritis that is thought to be autoummune or autoinflammatory. It has similar symptoms to systemic-onset juvenile idiopathic arthritis — fever, rash and joint pain. […] Inflammation may affect a few joints at first. Over time, more joints may be involved. […] Symptoms usually begin with a high fever that spikes once or twice a day and a salmon-pink rash on the trunk, arms or legs. Other symptoms include sore throat and swollen lymph nodes in the neck. […] A few weeks after these initial symptoms, joints and muscles begin aching. These aches last at least two weeks. The most commonly affected joints are the knee and wrist. The ankles, shoulders, elbows and finger joints may also be involved.

• #28 Adult Still disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/symptoms-causes/syc-20351907

  Muscular pain usually comes and goes with the fever. The pain can be severe enough to disrupt daily activities. […] Symptoms of this disorder can differ from person to person. They can mimic those of other conditions, including lupus and a type of cancer called lymphoma. […] Adult Still disease inflames the organs and joints. Most complications from the disease result from this inflammation. […] The chronic swelling and irritation that occurs with adult Still disease can damage the joints. The most commonly involved joints are the knees and wrists. Sometimes other joints, including the neck, foot, finger and hip joints, also are affected.

• #29 Adult Still Disease – UF Health

  https://ufhealth.org/conditions-and-treatments/adult-still-disease

  Adult Still disease (ASD) is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis. […] Almost all people with the disease will have fever, joint pain, sore throat, and a rash. […] Joint pain, warmth, and swelling are common. Most often, several joints are involved at the same time. Often, people with the condition have morning stiffness of joints that lasts for at least one hour. […] The fever comes on quickly once per day, most commonly in the afternoon or evening. […] The skin rash is often salmon-pink colored and comes and goes with the fever. […] Symptoms continue for a long time (chronic) in about one third of people with adult Still disease.

• #30 Adult Still’s Disease

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

  This rare type of inflammatory arthritis is marked by fever, rash and joint pain. […] Adult-onset Stills disease is a rare type of arthritis that is thought to be autoummune or autoinflammatory. It has similar symptoms to systemic-onset juvenile idiopathic arthritis — fever, rash and joint pain. […] Inflammation may affect a few joints at first. Over time, more joints may be involved. […] Symptoms usually begin with a high fever that spikes once or twice a day and a salmon-pink rash on the trunk, arms or legs. Other symptoms include sore throat and swollen lymph nodes in the neck. […] A few weeks after these initial symptoms, joints and muscles begin aching. These aches last at least two weeks. The most commonly affected joints are the knee and wrist. The ankles, shoulders, elbows and finger joints may also be involved.

• #31 Inflammatory Disorders: Adult Onset Still’s Disease | HSS

  https://www.hss.edu/condition-list_adult-onset-stills-disease.asp

  Adult-onset Stills disease is a rare immune-mediated, multisystem inflammatory disorder indicated by the Stills triad of high spiking fevers, rash and arthritis (joint pain). […] Characteristic symptoms include daily spiking fevers, evanescent (or short term) rashes, and arthritis, each occurring in about 75 to 95 percent of patients. […] Other less common, but more serious symptoms can include myalgia, inflammatory myopathy, liver abnormalities, pleuritis, pericarditis, splenomegaly, pericardial tamponade, myocarditis, pulmonary fibrosis, pleural effusions, adult respiratory distress syndrome, interstitial nephritis, subacute glomerulitis, renal amyloidosis, collapsing glomerulopathy, thrombotic thrombocytopenic purpura, pure red cell aplasia, cranial nerve palsies, seizures, aseptic meningoencephalitis, and Miller-Fisher syndrome. […] Life-threatening complications such as macrophage activation syndrome may occur and need to be diagnosed and treated promptly.

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

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

  c) Salmon-pink macular or maculopapular rash (80%; often transient, develops only during episodes of fever, rarely pruritic, most frequently located on the trunk and proximal limbs, less often on the face; it may be triggered by heat [eg, following a hot bath] or skin abrasion [eg, scratchy clothing]). […] d) Arthralgia (90%; worsens during febrile periods; occasionally arthritis, most commonly affecting the knees and wrists; approximately one-fourth of patients develop ankyloses of the involved joints). […] e) Myalgia (80%). […] f) Lymphadenopathy (50%; most frequently affecting the cervical lymph nodes; the lymph nodes may be tender; retroperitoneal lymphadenopathy may cause abdominal pain that is difficult to diagnose). […] g) Splenomegaly, hepatomegaly (50%). […] 2) Less frequent: Symptoms of pleuritis or pericarditis, weight loss.

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

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

  The primary symptoms include: Fever. When symptoms are active, most people have a daily fever with two spikes, one in the morning and one in the evening. Fever spikes are typically over 102 degrees Fahrenheit (39 degrees Celsius). Skin rash. A pink or salmon-colored rash usually appears when your fever is spiking. It may disappear in between spikes. It usually affects your chest, arms and legs first, but may spread. Joint pain. Most people feel achy in their joints, and many also experience swelling and stiffness. It may start in a few joints at first, but after a while, you may start to feel it all over. Muscle pain. You may also feel achy in your muscles, especially when your fever is spiking. […] In most cases, AOSD will impact your quality of life more than your long-term health. Its uncomfortable and can interfere with day-to-day activities, but it wont necessarily do lasting damage to your body.

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

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

  Adult-onset Still disease (AOSD) is a disease occurring in persons aged 16 years and resembling a systemic form of juvenile idiopathic arthritis. It may be associated with fever, rash, lymphadenopathy, splenomegaly, serositis, and inflammation of multiple organs. AOSD develops after the age of 16. In adults the symptoms may be due to the first occurrence of the disease or a relapse. AOSD may follow a self-limiting (lasting 1 year), relapsing (relapses may be very frequent), or chronic (symptoms are continuously present throughout the year) course. In some patients significant joint destruction occurs. […] Symptoms (Table 1): […] 1) Most frequent: […] a) Fever (80% of cases; usually 39 degrees Celsius, most commonly in the evening or twice during a 24-h period). […] b) Sore throat with features of inflammation (50%; frequently develops several days or weeks prior to other symptoms).

• #35 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  The rash in AOSD is classically characterized as evanescent, displaying a salmon-colored, macular, or maculopapular appearance. […] Arthritis in AOSD may initially present as mild, transient, and oligoarticular, potentially progressing into severe, destructive, and symmetric polyarticular forms. […] Myalgia tends to exacerbate during fever spikes and can occasionally be severe and debilitating. […] Symmetrical slightly tender lymphadenopathy is reported in one-third to two-thirds of patients with AOSD, while splenomegaly is observed in one-third to one-half of patients. […] The course of AOSD generally aligns with 1 of 3 patterns: self-limited illness, intermittent flares, or chronic Still disease. Predictors for chronicity and unfavorable outcomes include the presence of erosive polyarthritis at the time of presentation and involvement of shoulders or hips.

• #36 Adult onset Still disease

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

  Intermittent high fever occurs in all patients. Temperatures may get up to 39 or 40C before rapidly returning to normal levels. These fever spikes often occur at nearly the same time every day and may be accompanied by a feeling of extreme fatigue. […] Skin rash develops in about of 95% of patients. Characteristics of the rash are: Rash comes and goes with the fever, Small, non-itchy spots or patches up to 5cm in diameter, Salmon-pink coloured, Usually appear on the limbs and trunk, but may occur on the face or neck. […] Other systemic symptoms include: Swollen lymph glands and/or enlarged spleen/liver in 85% of patients, Muscle pain coinciding with fever, Inflamed lungs (pleuritis) and/or surrounding heart (pericarditis) in 60% of patients, Marked increase in white blood cell count, Severe anaemia, Abdominal pain.

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

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

  c) Salmon-pink macular or maculopapular rash (80%; often transient, develops only during episodes of fever, rarely pruritic, most frequently located on the trunk and proximal limbs, less often on the face; it may be triggered by heat [eg, following a hot bath] or skin abrasion [eg, scratchy clothing]). […] d) Arthralgia (90%; worsens during febrile periods; occasionally arthritis, most commonly affecting the knees and wrists; approximately one-fourth of patients develop ankyloses of the involved joints). […] e) Myalgia (80%). […] f) Lymphadenopathy (50%; most frequently affecting the cervical lymph nodes; the lymph nodes may be tender; retroperitoneal lymphadenopathy may cause abdominal pain that is difficult to diagnose). […] g) Splenomegaly, hepatomegaly (50%). […] 2) Less frequent: Symptoms of pleuritis or pericarditis, weight loss.

• #38 Adult onset Still disease

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

  Intermittent high fever occurs in all patients. Temperatures may get up to 39 or 40C before rapidly returning to normal levels. These fever spikes often occur at nearly the same time every day and may be accompanied by a feeling of extreme fatigue. […] Skin rash develops in about of 95% of patients. Characteristics of the rash are: Rash comes and goes with the fever, Small, non-itchy spots or patches up to 5cm in diameter, Salmon-pink coloured, Usually appear on the limbs and trunk, but may occur on the face or neck. […] Other systemic symptoms include: Swollen lymph glands and/or enlarged spleen/liver in 85% of patients, Muscle pain coinciding with fever, Inflamed lungs (pleuritis) and/or surrounding heart (pericarditis) in 60% of patients, Marked increase in white blood cell count, Severe anaemia, Abdominal pain.

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

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

  Adult-onset Stills disease is an autoimmune condition. This means that the condition is caused by your body’s immune system. AOSD can cause pain and stiffness in your joints, as well as inflammation in other areas of your body. […] People who develop AOSD often have the following symptoms: a fever, a sore throat, arthritis or joint pain, a salmon-pink rash that comes and goes quickly. Some people may not have all these symptoms at once, which can make it difficult to diagnose the condition. The fever caused by AOSD usually goes up and down a few times during the day, usually going up in the afternoon or evening. But some people may have a fever all the time or have spikes in the fever in the mornings. […] People can also have pain in their muscles or joints, or unexpected weight loss before the fevers start. AOSD can also cause fatigue, which is an overwhelming feeling of tiredness that doesn’t always get better with sleep or rest. People with AOSD frequently experience joint pain, going on to develop arthritis. Arthritis causes pain, swelling and stiffness in the joints. AOSD most commonly affects the knees, wrists and ankles, but it can also affect the hands, feet, hips, elbows, shoulders and jaw.

• #40 Adult Onset Stills Disease | EdRheum

  https://edrheum.org/adult-onset-stills-disease/

  Adult onset stills disease (AOSD) is a rare acquired autoinflammatory disease. It is characterised by fever and signs and symptoms of inflammation in the body that are not due to infection or autoimmune causes. The course of the disease is variable, some people experience one single episode of inflammation, and others get chronic episodic inflammation. Common symptoms: Fever, often variable during the day, usually rising in the afternoon or evening. Fatigue. Loss of appetite. Sore throat, mouth ulcers. Skin rash, salmon pink over the trunk, arms and legs. Joint pain and swelling. Muscle pain (myalgia). Lymphadenopathy, swollen lymph nodes in the neck. AOSD usually follows one of 3 patterns: Self limiting- remission achieved within a year. Intermittent flares- recurrent flares of fevers and rash, often with addition of joint symptoms. Chronic- persistent active disease with predominantly joint problems.

• #41 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Stills Disease is a rare inflammatory disease that exists in two main forms: adult-onset Stills disease (AOSD), and a type of childhood arthritis called systemic juvenile idiopathic arthritis (SJIA). Symptoms include fatigue, fever, joint pain, and inflammation. […] Both the adult form and the juvenile form of Stills disease are marked by fatigue, fever, joint pain, and inflammation. A distinctive salmon-coloured rash is often present, and several of the body’s organs can be affected. […] The main symptoms of the condition include fatigue, muscle pain, sore throat, high fever reaching up to 41C, a salmon-pink rash, and inflammatory arthritis which tends to be more severe in the juvenile form. […] For the juvenile form of the disease, up to 30% of children with SJIA still experience symptoms 10 years after their first symptoms. Symptoms can persist into adulthood. AOSD generally follows one of three main patterns: Monophasic pattern: a single episode of symptoms that typically lasts weeks to months, but usually less than a year. Polyphasic (intermittent) pattern: more than one episode of symptoms. Patients usually remain free from symptoms for weeks to years between episodes. Chronic pattern: patients with chronic AOSD have persistent symptoms over time.

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

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

  The course of AOSD occurs in three main patterns: Monophasic: This refers to a single episode of symptoms that may last from weeks to months but persists for less than a year. Polyphasic, or intermittent: This describes when a person develops more than one episode of symptoms. The individual may be symptom-free for weeks to years between episodes, and subsequent episodes tend to be shorter and less severe. Chronic: In the chronic form, the symptoms persist over time. […] Adult-onset Stills disease is a rare autoimmune inflammatory condition characterized by symptoms of fever, joint pain, and salmon-pink rashes. The condition develops in adulthood and is similar to systemic juvenile idiopathic arthritis, which occurs in childhood. AOSD can have different patterns, meaning that some people only experience a single episode, whereas others have persistent symptoms.

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

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

  The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […] Around one-third of people diagnosed with the condition will have it for less than a year before their symptoms ease. They might need to stay on drug treatment for a bit longer to make sure the condition is completely under control. Another third of people will have flares of the condition before symptoms disappear completely. The flares can sometimes be years apart, and they don’t always affect the joints. Symptoms may get easier to manage in later flares.

• #44 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Many people with AOSD can live a full and normal life with the right treatment. In some cases, the condition can resolve spontaneously. Usually, however, AOSD tends to affect people in one of three ways: Around one-third of people with AOSD: Will have it for less than a year before their symptoms ease. They might need to stay on treatment for some time to make sure the condition is completely under control. Will have flares of the condition before symptoms disappear completely. The flares can be years apart, and may not affect the joints. Symptoms may become easier to manage. Will develop long-term arthritis and may have flare ups quite frequently and for a long time.

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

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

  The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […] Around one-third of people diagnosed with the condition will have it for less than a year before their symptoms ease. They might need to stay on drug treatment for a bit longer to make sure the condition is completely under control. Another third of people will have flares of the condition before symptoms disappear completely. The flares can sometimes be years apart, and they don’t always affect the joints. Symptoms may get easier to manage in later flares.

• #46 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Many people with AOSD can live a full and normal life with the right treatment. In some cases, the condition can resolve spontaneously. Usually, however, AOSD tends to affect people in one of three ways: Around one-third of people with AOSD: Will have it for less than a year before their symptoms ease. They might need to stay on treatment for some time to make sure the condition is completely under control. Will have flares of the condition before symptoms disappear completely. The flares can be years apart, and may not affect the joints. Symptoms may become easier to manage. Will develop long-term arthritis and may have flare ups quite frequently and for a long time.

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

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

  Around one-third of people with AOSD will go on to develop long-term arthritis and may have flares quite regularly and for a long time. In some people, AOSD can eventually cause permanent damage to the joints, usually if people have inflammation in their joints for a long time. The risk of this is reduced if the condition is brought under control by drugs. […] Many people with AOSD can live a full and normal life with the right treatment. Some of the symptoms of AOSD might make you feel more conscious of how you look. People with AOSD often comment on changes to their weight, as well as the look of their joints. Some people also experience increased sweating due to the fevers. […] The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […]

• #48 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Many people with AOSD can live a full and normal life with the right treatment. In some cases, the condition can resolve spontaneously. Usually, however, AOSD tends to affect people in one of three ways: Around one-third of people with AOSD: Will have it for less than a year before their symptoms ease. They might need to stay on treatment for some time to make sure the condition is completely under control. Will have flares of the condition before symptoms disappear completely. The flares can be years apart, and may not affect the joints. Symptoms may become easier to manage. Will develop long-term arthritis and may have flare ups quite frequently and for a long time.

• #49 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  The rash in AOSD is classically characterized as evanescent, displaying a salmon-colored, macular, or maculopapular appearance. […] Arthritis in AOSD may initially present as mild, transient, and oligoarticular, potentially progressing into severe, destructive, and symmetric polyarticular forms. […] Myalgia tends to exacerbate during fever spikes and can occasionally be severe and debilitating. […] Symmetrical slightly tender lymphadenopathy is reported in one-third to two-thirds of patients with AOSD, while splenomegaly is observed in one-third to one-half of patients. […] The course of AOSD generally aligns with 1 of 3 patterns: self-limited illness, intermittent flares, or chronic Still disease. Predictors for chronicity and unfavorable outcomes include the presence of erosive polyarthritis at the time of presentation and involvement of shoulders or hips.

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

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

  3) Rare: Pulmonary fibrosis, myocarditis or cardiac tamponade, hair loss, Sjgren syndrome, macrophage activation syndrome (MAS) (a form of hemophagocytic lymphohistiocytosis [HLH]), aseptic meningitis, peripheral neuropathy, amyloidosis, subacute glomerulonephritis and interstitial nephritis, hemolytic anemia, disseminated intravascular coagulation, cataract, hearing impairment. […] The natural history of AOSD can be quite variable. Some patients have a single systemic self-limited episode with symptoms improving within a few months and resolving by 1 year, while other patients have a polycyclic form of the disease, with multiple flares of the systemic and arthritic symptoms and clear periods of remission between the flares. Finally, the remaining patients have a form associated with chronic disease, where inflammatory arthritis is the predominant manifestation. These patients are prone to develop secondary osteoarthritis, with an associated impact on health-related quality of life and disability.

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

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

  Predictive factors for each subset’s evolution have been identified. Female gender, proximal arthritis at disease onset, thrombocytosis, and steroid dependence represent the articular pattern. […] The systemic subset appears to be associated with high fever (>39 °C), high levels of liver enzymes or acute phase reactants, thrombocytopenia, and hyperferritinemia. […] The prognosis for AOSD is favorable, with an estimated specific mortality rate of 1–3%. […] Some patients, however, experience complications. […] The most severe complication of AOSD is macrophage activation syndrome (MAS). […] High fever, hepatosplenomegaly, cytopenias, coagulopathy, extreme hyperferritinemia, and hemophagocytosis on bone marrow aspirates are the most common symptoms of MAS.

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

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

  Predictive factors for each subset’s evolution have been identified. Female gender, proximal arthritis at disease onset, thrombocytosis, and steroid dependence represent the articular pattern. […] The systemic subset appears to be associated with high fever (>39 °C), high levels of liver enzymes or acute phase reactants, thrombocytopenia, and hyperferritinemia. […] The prognosis for AOSD is favorable, with an estimated specific mortality rate of 1–3%. […] Some patients, however, experience complications. […] The most severe complication of AOSD is macrophage activation syndrome (MAS). […] High fever, hepatosplenomegaly, cytopenias, coagulopathy, extreme hyperferritinemia, and hemophagocytosis on bone marrow aspirates are the most common symptoms of MAS.

• #53

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

  Conversely, patients with elderly onset ( 65 years) had less skin rash, sore throat, myalgia, hepatosplenomegaly and overall Pouchots score compared with adults ( 65 years). […] Furthermore, different subsets of AOSD were recently identified depending on clinical/laboratory features and on the basis of the different expressions of pro-inflammatory cytokines. […] AOSD was historically subdivided into three different phenotypes depending on the evolution over time: the systemic pattern characterised by recurrent episodes of inflammation, the monocyclic pattern that presents only once in a lifetime and the chronic articular pattern that is predominantly characterised by joint involvement. […] The treatment of AOSD varies according to the pattern and the clinical manifestations exhibited by patients; generally, the first-line therapy relies on the employment of traditional drugs such as non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids (GCs); most of the data on the efficacy of conventional synthetic (cs) disease-modifying anti-rheumatic drugs (DMARDs) come from longitudinal studies, and csDMARDs have proven to be more effective at controlling articular manifestations.

• #54 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  Complications of AOSD include MAS, amyloidosis, disseminated intravascular coagulopathy (DIC), pulmonary arterial hypertension (PAH), thrombotic thrombocytopenic purpura (TTP), and diffuse alveolar hemorrhage. Timely recognizing and managing these complications is crucial in optimizing patient outcomes and minimizing potential long-term sequelae.

• #55 Inflammatory Disorders: Adult Onset Still’s Disease | HSS

  https://www.hss.edu/condition-list_adult-onset-stills-disease.asp

  Adult-onset Stills disease is a rare immune-mediated, multisystem inflammatory disorder indicated by the Stills triad of high spiking fevers, rash and arthritis (joint pain). […] Characteristic symptoms include daily spiking fevers, evanescent (or short term) rashes, and arthritis, each occurring in about 75 to 95 percent of patients. […] Other less common, but more serious symptoms can include myalgia, inflammatory myopathy, liver abnormalities, pleuritis, pericarditis, splenomegaly, pericardial tamponade, myocarditis, pulmonary fibrosis, pleural effusions, adult respiratory distress syndrome, interstitial nephritis, subacute glomerulitis, renal amyloidosis, collapsing glomerulopathy, thrombotic thrombocytopenic purpura, pure red cell aplasia, cranial nerve palsies, seizures, aseptic meningoencephalitis, and Miller-Fisher syndrome. […] Life-threatening complications such as macrophage activation syndrome may occur and need to be diagnosed and treated promptly.

• #56 Adult Still disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/adult-stills-disease/symptoms-causes/syc-20351907

  Muscular pain usually comes and goes with the fever. The pain can be severe enough to disrupt daily activities. […] Symptoms of this disorder can differ from person to person. They can mimic those of other conditions, including lupus and a type of cancer called lymphoma. […] Adult Still disease inflames the organs and joints. Most complications from the disease result from this inflammation. […] The chronic swelling and irritation that occurs with adult Still disease can damage the joints. The most commonly involved joints are the knees and wrists. Sometimes other joints, including the neck, foot, finger and hip joints, also are affected.

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

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

  Around one-third of people with AOSD will go on to develop long-term arthritis and may have flares quite regularly and for a long time. In some people, AOSD can eventually cause permanent damage to the joints, usually if people have inflammation in their joints for a long time. The risk of this is reduced if the condition is brought under control by drugs. […] Many people with AOSD can live a full and normal life with the right treatment. Some of the symptoms of AOSD might make you feel more conscious of how you look. People with AOSD often comment on changes to their weight, as well as the look of their joints. Some people also experience increased sweating due to the fevers. […] The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […]

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

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

  3) Rare: Pulmonary fibrosis, myocarditis or cardiac tamponade, hair loss, Sjgren syndrome, macrophage activation syndrome (MAS) (a form of hemophagocytic lymphohistiocytosis [HLH]), aseptic meningitis, peripheral neuropathy, amyloidosis, subacute glomerulonephritis and interstitial nephritis, hemolytic anemia, disseminated intravascular coagulation, cataract, hearing impairment. […] The natural history of AOSD can be quite variable. Some patients have a single systemic self-limited episode with symptoms improving within a few months and resolving by 1 year, while other patients have a polycyclic form of the disease, with multiple flares of the systemic and arthritic symptoms and clear periods of remission between the flares. Finally, the remaining patients have a form associated with chronic disease, where inflammatory arthritis is the predominant manifestation. These patients are prone to develop secondary osteoarthritis, with an associated impact on health-related quality of life and disability.

• #59 Adult Onset Stills Disease | EdRheum

  https://edrheum.org/adult-onset-stills-disease/

  Rare complications: Heart, pericarditis- inflammation of the heart lining causing chest pain. Lungs, pleuritis- inflammation of the lung lining, often leading to fluid build up around the lung (pleural effusion) resulting in chest pain and shortness of breath. Interstitial lung disease- scarring in the lung leading to cough and breathlessness. Abdomen, peritonitis- inflammation of the abdominal wall lining causing pain. Colitis- inflammation of the bowel lining leading to diarrhoea, nausea and vomiting. Enlarged liver or spleen. Macrophage activation syndrome, a life threatening complication with high blood ferritin levels, low white blood cells, clotting abnormalities and deranged liver function. Usually triggered by a viral infection.

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

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

  Predictive factors for each subset’s evolution have been identified. Female gender, proximal arthritis at disease onset, thrombocytosis, and steroid dependence represent the articular pattern. […] The systemic subset appears to be associated with high fever (>39 °C), high levels of liver enzymes or acute phase reactants, thrombocytopenia, and hyperferritinemia. […] The prognosis for AOSD is favorable, with an estimated specific mortality rate of 1–3%. […] Some patients, however, experience complications. […] The most severe complication of AOSD is macrophage activation syndrome (MAS). […] High fever, hepatosplenomegaly, cytopenias, coagulopathy, extreme hyperferritinemia, and hemophagocytosis on bone marrow aspirates are the most common symptoms of MAS.

• #61 Inflammatory Disorders: Adult Onset Still’s Disease | HSS

  https://www.hss.edu/condition-list_adult-onset-stills-disease.asp

  Adult-onset Stills disease is a rare immune-mediated, multisystem inflammatory disorder indicated by the Stills triad of high spiking fevers, rash and arthritis (joint pain). […] Characteristic symptoms include daily spiking fevers, evanescent (or short term) rashes, and arthritis, each occurring in about 75 to 95 percent of patients. […] Other less common, but more serious symptoms can include myalgia, inflammatory myopathy, liver abnormalities, pleuritis, pericarditis, splenomegaly, pericardial tamponade, myocarditis, pulmonary fibrosis, pleural effusions, adult respiratory distress syndrome, interstitial nephritis, subacute glomerulitis, renal amyloidosis, collapsing glomerulopathy, thrombotic thrombocytopenic purpura, pure red cell aplasia, cranial nerve palsies, seizures, aseptic meningoencephalitis, and Miller-Fisher syndrome. […] Life-threatening complications such as macrophage activation syndrome may occur and need to be diagnosed and treated promptly.

• #62

  https://www.aiarthritis.org/stillsdisease

  Inflammatory Arthritis – Historically arthritis, or joint involvement, was considered one of the major Still’s Disease symptoms. However, recent research suggests about 25% of patients may not have arthritis at or near onset – some may not develop it for years, if ever. In others, the arthritis is severe. […] Organ Involvement – Generalized lymph nodes/glandular swelling. Enlargement of the liver and/or spleen. Inflammation of lungs (pleuritis), the saclike covering of your heart (pericarditis), the muscular portion of your heart (myocarditis), eyes, serous tissues. Organ involvement may be present at onset. […] Other Complications – Macrophage activation syndrome (MAS) – a massive inflammatory response that overwhelms the whole body, including organs. MAS is more common in SJIA and may be present at onset. […] Mental health – A quarter of patients surveyed reported mental health issues including: irritability, low self esteem, and difficulty coping with stress, anxiety, isolation, frustration, and depression.

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

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

  3) Rare: Pulmonary fibrosis, myocarditis or cardiac tamponade, hair loss, Sjgren syndrome, macrophage activation syndrome (MAS) (a form of hemophagocytic lymphohistiocytosis [HLH]), aseptic meningitis, peripheral neuropathy, amyloidosis, subacute glomerulonephritis and interstitial nephritis, hemolytic anemia, disseminated intravascular coagulation, cataract, hearing impairment. […] The natural history of AOSD can be quite variable. Some patients have a single systemic self-limited episode with symptoms improving within a few months and resolving by 1 year, while other patients have a polycyclic form of the disease, with multiple flares of the systemic and arthritic symptoms and clear periods of remission between the flares. Finally, the remaining patients have a form associated with chronic disease, where inflammatory arthritis is the predominant manifestation. These patients are prone to develop secondary osteoarthritis, with an associated impact on health-related quality of life and disability.

• #64 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  Complications of AOSD include MAS, amyloidosis, disseminated intravascular coagulopathy (DIC), pulmonary arterial hypertension (PAH), thrombotic thrombocytopenic purpura (TTP), and diffuse alveolar hemorrhage. Timely recognizing and managing these complications is crucial in optimizing patient outcomes and minimizing potential long-term sequelae.

• #65 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. […] The overall prognosis is generally good, but acute life-threatening manifestations may occur in rare cases. The most classical complication is macrophage activation syndrome but blood coagulation disorders, fulminant hepatitis, cardiac and pulmonary complications may occur. Some patients with chronic disease and major joint involvement may have significantly altered quality of life, but this tends to disappear thanks to a better and earlier management with biologics.

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

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

  Prognosis is usually favorable but manifestations of the disease affecting the lungs, heart, or kidneys may occasionally cause severe life-threatening complications. […] One set of 21 adult-onset Still’s disease patients were divided into four types, according to clinical course patterns. These included monocyclic systemic disease, polycyclic systemic disease, chronic articular monocyclic systemic disease, and chronic articular polycyclic systemic disease. People with chronic articular and polyarticular disease were at higher risk to develop disabling arthritis.

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

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

  Around one-third of people with AOSD will go on to develop long-term arthritis and may have flares quite regularly and for a long time. In some people, AOSD can eventually cause permanent damage to the joints, usually if people have inflammation in their joints for a long time. The risk of this is reduced if the condition is brought under control by drugs. […] Many people with AOSD can live a full and normal life with the right treatment. Some of the symptoms of AOSD might make you feel more conscious of how you look. People with AOSD often comment on changes to their weight, as well as the look of their joints. Some people also experience increased sweating due to the fevers. […] The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […]

• #68 Still’s Disease Facts | 7 Surprising Facts About Still’s Disease

  https://resources.healthgrades.com/right-care/arthritis/7-surprising-facts-about-stills-disease

  Although Still’s disease is classified as a type of arthritis, the condition also affects most of the internal organs, including the heart, lungs, liver and spleen. The excessive inflammation generated by Still’s disease can cause these organs to become enlarged, resulting in organ malfunction. In the case of the liver, Still’s disease often causes certain liver enzymes to become elevated—a data point used to achieve a diagnosis. Still’s disease treatment focuses on reducing systemic inflammation to help protect the organs as well as the joints. […] For approximately two-thirds of people who develop Still’s disease, the condition goes into remission after one episode or several cyclical episodes over the course of a few years. But the remaining one-third of people diagnosed with Still’s develop a chronic form of the disorder and can face a life-threatening complication called macrophage activation syndrome (MAS). MAS causes an extreme proliferation of macrophages (a type of large immune system cell) that overrun the body and can result in death. For most people, life expectancy in Still’s disease is normal, but for a few people the condition can be life-limiting.

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

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

  Predictive factors for each subset’s evolution have been identified. Female gender, proximal arthritis at disease onset, thrombocytosis, and steroid dependence represent the articular pattern. […] The systemic subset appears to be associated with high fever (>39 °C), high levels of liver enzymes or acute phase reactants, thrombocytopenia, and hyperferritinemia. […] The prognosis for AOSD is favorable, with an estimated specific mortality rate of 1–3%. […] Some patients, however, experience complications. […] The most severe complication of AOSD is macrophage activation syndrome (MAS). […] High fever, hepatosplenomegaly, cytopenias, coagulopathy, extreme hyperferritinemia, and hemophagocytosis on bone marrow aspirates are the most common symptoms of MAS.

• #70 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  Of these patients, 78% were female, and the median age at onset was 34 (25-49) years [median (25th-75th percentile)]. The median follow-up time was 7 (3-10) years [median (25th-75th percentile)]. Clinical manifestations at admission were used to subdivide patients with AOSD as follows: systemic inflammation (cluster 1), pure (cluster 2), and intermediate (cluster 3). Each subtype had distinct clinical manifestations and prognoses: cluster 1 (34.6%) multiple organ manifestations, highest infection rate and mortality, and more than half of the patients with at least one relapse during follow-up; cluster 2 (21.3%) exclusively female, rash and joint involvement, no internal organ involvement, no mortality, and most of the patients with a monocyclic course; and cluster 3 (44.1%) less infection rate, no serious complications, and lower mortality rate. The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. Independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.10-21.89), hepatomegaly (HR: 5.05, 95% CI: 1.44-17.70), infection (HR: 15.56, 95% CI: 5.88-41.20), and MAS (HR: 26.82, 95% CI: 7.52-95.60).

• #71 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  Of these patients, 78% were female, and the median age at onset was 34 (25-49) years [median (25th-75th percentile)]. The median follow-up time was 7 (3-10) years [median (25th-75th percentile)]. Clinical manifestations at admission were used to subdivide patients with AOSD as follows: systemic inflammation (cluster 1), pure (cluster 2), and intermediate (cluster 3). Each subtype had distinct clinical manifestations and prognoses: cluster 1 (34.6%) multiple organ manifestations, highest infection rate and mortality, and more than half of the patients with at least one relapse during follow-up; cluster 2 (21.3%) exclusively female, rash and joint involvement, no internal organ involvement, no mortality, and most of the patients with a monocyclic course; and cluster 3 (44.1%) less infection rate, no serious complications, and lower mortality rate. The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. Independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.10-21.89), hepatomegaly (HR: 5.05, 95% CI: 1.44-17.70), infection (HR: 15.56, 95% CI: 5.88-41.20), and MAS (HR: 26.82, 95% CI: 7.52-95.60).

• #72 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  The results showed that patients with the systemic inflammation type of disease had the worst prognosis; the survival rate was significantly lower than that of patients with the pure and intermediate types of disease. In addition, pure-type patients had the highest rate of non-recurrence during follow-up, and systemic inflammatory patients had the highest rate of recurrence. Finally, we predicted that the mortality-associated factors were age at onset 50, infection, hepatomegaly, and MAS, which might be valuable for clinical use.

• #73 Still’s Disease: Life Expectancy – Klarity Health Library

  https://my.klarity.health/stills-disease-life-expectancy/

  Adult-Onset Stills Disease (AOSD) is a rare form of inflammatory arthritis that is characterised by fevers, rashes, and joint pain. A fever of at least 102 F (38.9 C) may occur on a daily basis for a week or more. Individuals may experience peaks in temperature in the later afternoon or early in the evening, with two fever spikes daily. […] The prognosis for AOSD is generally good; the symptoms can be managed, and the condition is not fatal. However, some complications of AOSD, such as macrophage activation syndrome, can be fatal. […] The relapse rate of the condition is high. (For example, Appenzeller et al. report that around 75% of patients relapsed in 6.9 years of follow-up). […] Complications of AOSD can be fatal, however. The inflammation caused by AOSD can build up around the lungs, making deep breathing difficult. Another complication of AOSD, macrophage activation syndrome, results in low blood cell counts, high levels of triglyceride, and abnormal liver functioning. This complication is rare but can lead to death. […] How long the condition may persist varies between individuals. However, some research suggests that the average duration of AOSD is a decade, with over half of patients continuing to require medication even ten years post-diagnosis.

• #74 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  The results showed that patients with the systemic inflammation type of disease had the worst prognosis; the survival rate was significantly lower than that of patients with the pure and intermediate types of disease. In addition, pure-type patients had the highest rate of non-recurrence during follow-up, and systemic inflammatory patients had the highest rate of recurrence. Finally, we predicted that the mortality-associated factors were age at onset 50, infection, hepatomegaly, and MAS, which might be valuable for clinical use.

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

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

  Around one-third of people with AOSD will go on to develop long-term arthritis and may have flares quite regularly and for a long time. In some people, AOSD can eventually cause permanent damage to the joints, usually if people have inflammation in their joints for a long time. The risk of this is reduced if the condition is brought under control by drugs. […] Many people with AOSD can live a full and normal life with the right treatment. Some of the symptoms of AOSD might make you feel more conscious of how you look. People with AOSD often comment on changes to their weight, as well as the look of their joints. Some people also experience increased sweating due to the fevers. […] The symptoms of AOSD can start suddenly or gradually. When symptoms start suddenly, this is known as a flare. This often happens for no particular reason. Flares can vary in how badly they affect people with AOSD, and they can be very frequent or may be years apart. Some people with AOSD only experience the symptoms for a short period, but for others the condition can return or continue causing symptoms for a long time. […]

• #76

  https://www.aiarthritis.org/stillsdisease

  Inflammatory Arthritis – Historically arthritis, or joint involvement, was considered one of the major Still’s Disease symptoms. However, recent research suggests about 25% of patients may not have arthritis at or near onset – some may not develop it for years, if ever. In others, the arthritis is severe. […] Organ Involvement – Generalized lymph nodes/glandular swelling. Enlargement of the liver and/or spleen. Inflammation of lungs (pleuritis), the saclike covering of your heart (pericarditis), the muscular portion of your heart (myocarditis), eyes, serous tissues. Organ involvement may be present at onset. […] Other Complications – Macrophage activation syndrome (MAS) – a massive inflammatory response that overwhelms the whole body, including organs. MAS is more common in SJIA and may be present at onset. […] Mental health – A quarter of patients surveyed reported mental health issues including: irritability, low self esteem, and difficulty coping with stress, anxiety, isolation, frustration, and depression.

• #77

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

  The use of biological (b) DMARDs instead represents a valid option for treating both systemic and articular manifestations, providing satisfactory results in terms of safety and efficacy, despite data derived mostly from clinical trials involving children with sJIA. […] In AOSD, both innate and adaptive immunity are upregulated, contributing to the clinical manifestations presented. […] The pathophysiology of AOSD is known to be based on an imbalance between innate and adaptive immunity and is characterised by a substantial increase in pro-inflammatory cytokines that contribute to disease progression. […] The final result of the immunological imbalance is the massive release of pro-inflammatory cytokines and the exuberant production of mediators that may amplify and perpetuate, including IL-1, IL-6, IL-8, IL-17, TNF- and IFN-; when the production is uncontrolled, the exacerbation of this inflammatory upregulation may provoke the so-called cytokine storm. […] The most recent advances, as well as the introduction of biologic drugs, have significantly improved the quality of life of patients with AOSD.

Zobacz więcej