Materiały źródłowe

• #1 6 Vasculitis Medications To Help Manage the Disease

  https://health.clevelandclinic.org/vasculitis-medications

  Treatments have improved outcomes for a condition once viewed as fatal […] Various medications can be used to ease the inflammation that vasculitis brings to blood vessels. As that swelling decreases, blood flows better through your system, reducing health risks. […] But with medication, vasculitis can now be managed as a chronic condition and that’s a significant step forward from the old days. […] Treatment and medication options to address and reduce that swelling vary case by case. […] Medications typically fall into one of two categories: Corticosteroids and Disease-modifying antirheumatic drugs (DMARDs). […] Almost all types of vasculitis get treated with a specific class of corticosteroids known as glucocorticoids. For certain types of vasculitis, another medication typically a DMARD may be used in tandem.

• #1 General Vasculitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/general-vasculitis/

  Treatment is based on numerous factors including the specific type of vasculitis, symptoms, organs affected, disease severity, laboratory test results, age, overall health and more. It is essential to work closely with your doctor in developing a comprehensive treatment plan. […] Treatment usually involves two phases: controlling the inflammation to achieve remission, and maintenance treatment to prevent relapses. Common treatments include the following: […] Glucocorticoids such as prednisone are often the first line of treatment for vasculitis to reduce inflammation. Glucocorticoids are also immunosuppressive medications. […] For more serious forms of vasculitis, other medications that suppress the immune system are often prescribed including methotrexate, azathioprine, mycophenolate mofetil, and cyclophosphamide.

• #1 Vasculitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vasculitis/diagnosis-treatment/drc-20363485

  Treatment aims to control the swelling and irritation, called inflammation. It also aims to manage the conditions that may be causing the vasculitis. […] A corticosteroid medicine, such as prednisone (Rayos), is the most common type of medicine prescribed to control the inflammation of vasculitis. […] Your healthcare professional may add other medicines to corticosteroids to control the inflammation. That way, you can lower the dose of corticosteroids more quickly. The medicine used depends on the type of vasculitis. Medicines may include methotrexate (Trexall), azathioprine (Imuran, Azasan), mycophenolate mofetil (CellCept), cyclophosphamide (Cytoxan), tocilizumab (Actemra) or rituximab (Rituxan). […] Sometimes, vasculitis causes a balloonlike bulge in the wall of a blood vessel. This is called an aneurysm. This bulge may need treatment with surgery to lower the risk of it breaking. Blocked arteries also may need surgical treatment to restore blood flow to the affected area.

• #1 Vasculitis – Treatment | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/vasculitis/treatment

  The goal of vasculitis treatment is usually to slow down the body’s inflammatory attack on your blood vessels. People who have mild vasculitis may find relief with over-the-counter pain medicines. For severe vasculitis, you may be prescribed medicines. With treatment, vasculitis can go into remission, which is a period of time when you don’t have symptoms. […] Over-the-counter pain medicines can relieve symptoms of mild vasculitis. For more serious cases, your healthcare provider may prescribe medicines. […] Corticosteroids lower the activity of the body’s defense system in your blood vessels. For some types of vasculitis, you will need steroids for months or years. Corticosteroids can lower your bone density, raise your blood sugar and blood pressure levels and cause your skin to get thinner.

• #1 Dr. Patrick Nachman & Treating ANCA Vasculitis with Rituximab | Department of Medicine

  https://www.med.unc.edu/medicine/news/chairs-corner/podcast/treating-with-rituximab/

  Dr. Patrick Nachman and Dr. Ron Falk discuss these details in this podcast. […] The classic approach to starting therapy for an aggressive form of vasculitis involving the lung and kidney-or the kidney alone, typically it starts with what we call an induction phase of treatment which usually includes using relatively large doses of corticosteroids by vein and then by mouth. […] More recently, over the last several years, rituximab has been introduced as an option for the induction phase of treatment of vasculitis. […] Rituximab has been introduced as an option several years ago and it has been pushed to the forefront by a large randomized control trial called the RAVE trial, where patients with ANCA vasculitis were randomized to a treatment of rituximab versus the more traditional approach of using cyclophosphamide.

• #1 Overview of Vasculitis – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/overview-of-vasculitis

  Less severe presentations of vasculitis may be managed with lower doses of corticosteroids, along with less potent immunosuppressants. […] Corticosteroids are tapered off or to the lowest dose that can maintain remission. For several forms of vasculitis, a less toxic immunosuppressant is prescribed to maintain remission as the corticosteroids are decreased and ideally eliminated. […] Long-term use of corticosteroids can have significant adverse effects. Patients who are taking 7.5 mg of prednisone daily or equivalent doses of other corticosteroids should be given calcium and vitamin D supplements and bisphosphonates to help prevent or minimize osteoporosis; bone density monitoring should be considered.

• #1

  https://rheumatology.org/patients/vasculitis

  Glucocorticoids (Deltasone) are used to treat inflammation in many types of vasculitis. For milder vasculitis, methotrexate (Rheumatrex), azathioprine (Imuran) and other immunosuppressants may be used. Newer biologic drugs can treat certain types of vasculitis, such as rituximab (Rituxan) or avacopan (Tavenos) for GPA/MPA; tocilizumab (Actemra) for GCA; mepolizumab (Nucala) or Benralizumab (Fasenra) for EGPA; and apremilast (Otezla) for Bechets disease. Cyclophosphamide (Cytoxan) is an immunosuppressant used for life threatening disease. Some patients with severe disease receive treatments like plasma exchange (plasmapharesis) or intravenous immunoglobulin (IVIG). If severe vasculitis damages blood vessels or organs, surgery may be needed to repair them. This may include vascular bypass grafting, sinus surgery or kidney transplant. […] With treatments, the outlook for patients with vasculitis is good.

• #1

  https://link.springer.com/article/10.1007/s40674-016-0045-8

  The use of cyclophosphamide and glucocorticoids as induction therapy for AAV has improved the survival rates from 20 % to well over 80 % at 2 years. […] Rituximab, a B cell-depleting agent, in the last decade has been shown to be non-inferior to cyclophosphamide and is now licensed for use of remission induction. Currently, strategies to minimise glucocorticoid exposure are also being explored. […] Treatment of AAV typically includes two distinct phases, an induction phase (3 to 6 months), to gain rapid control of disease activity, and a maintenance phase (18 to 24 months), to maintain remission and prevent relapses, using less toxic agents. […] Cyclophosphamide, an alkylating agent, inhibits DNA replication by alkylating guanidine nucleotides. […] Cyclophosphamide is usually given either as oral or pulsed therapy for 3 to 6 months and is replaced by less toxic drugs after achieving remission.

• #1 New ANCA-Associated Vasculitis treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=101

  Cyclophosphamide, a potent immunosuppressive agent, is highly effective in inducing remission, especially in severe cases involving kidney or lung damage. […] Rituximab, a monoclonal antibody targeting B-cells, is FDA-approved for induction and maintenance therapy in AAV and offers similar efficacy with potentially fewer long-term side effects. […] Azathioprine and methotrexate are commonly used as maintenance therapies following induction with cyclophosphamide or rituximab. […] Tavneos (avacopan) is a newly FDA-approved medication specifically indicated for ANCA-associated vasculitis. […] Rituximab is a monoclonal antibody that targets CD20-positive B cells, reducing antibody production and inflammation. […] Cyclophosphamide is a potent immunosuppressive agent traditionally used for severe AAV cases, particularly those involving major organs such as kidneys or lungs.

• #1 Stay the Course: How to Maintain Remission in Vasculitis – The Rheumatologist

  https://www.the-rheumatologist.org/article/stay-the-course-how-to-maintain-remission-in-vasculitis/

  With regard to cyclophosphamide, the Cyclophosphamide vs. Azathioprine for Early Remission Phase of Vasculitis (CYCAZAREM) trial demonstrated that in patients with generalized vasculitis, withdrawal of cyclophosphamide and substitution of azathioprine after remission did not increase the rate of relapse. This finding indicates that extended treatment with cyclophosphamide is not necessary for maintenance of remission. […] With regard to methotrexate and azathioprine for maintenance of remission in ANCA-associated vasculitis, the two medications were shown to be similar in efficacy in the Wegeners GranulomatosisEntretien (WEGENT) trial.

• #1 Treatment of ANCA Vasculitis | ANCA Vasculitis NewsEnvelope icon

  https://ancavasculitisnews.com/treatment-of-anca-vasculitis/

  Maintainance therapies generally use an immunosuppressant, a drug which reduces the immune response. Commonly used maintenance therapies include azathioprine and methotrexate. If the patient is intolerant or not responding to these treatments, other options include Arava (leflunomide) and CellCept (mycophenolate mofetil). These are often used in combination with glucocorticoids. […] Azathioprine, especially, is used as a maintenance therapy for remission in eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome. […] Methotrexate or CellCept may be used in combination to induce remission in cases of mild non-organ-threatening ANCA vasculitis.

• #1 Treatment of Vasculitis: Beyond the Basics | IntechOpen

  https://www.intechopen.com/chapters/72404

  Vasculitis is the inflammation of blood vessels in the human body. It causes changes and remodeling in the walls of the vessels that include thickening, narrowing and scarring. As a result, the blood flow to the organs and tissues gets restricted leading to organ damage. The cause of primary vasculitis is not known; however, most cases are thought to be autoimmune. In the present era, it is getting difficult to treat vasculitis with conventional therapies, which includes cyclophosphamide, methotrexate, azathioprine and mycophenolate mofetil, with increasing rates of relapses. Since ever, corticosteroids and cytotoxic agents or immunosuppressants have been the mainstay for treating systemic vasculitis. However, the introduction of newer biological agents have bring about a revolution in the treatment of relapses and in cases where there is failure to induce and sustain remission.

• #1 Treatment of ANCA Vasculitis | ANCA Vasculitis NewsEnvelope icon

  https://ancavasculitisnews.com/treatment-of-anca-vasculitis/

  Cytoxan (cyclophosphamide) was originally approved as a cancer therapy to block cell growth. In ANCA vasculitis, it is believed to kill the immune cells (such as neutrophils) that cause the damaging inflammation. It has been shown to be effective at inducing remission in 90 percent of ANCA vasculitis patients, but can cause severe side effects especially if used long-term. […] Rituxan (rituximab) is an antibody or protein designed to block a specific target. By inhibiting a protein called CD20, Rituxan reduces the numbers of B-cells. B-cells are a type of immune cells that are involved in producing ANCA, the trigger for the immune system to start attacking the blood vessels. […] Plasma exchange, or PEX, involves taking blood from the patient, separating the plasma, and returning the blood cells to the patient in a substitute fluid.

• #1 New Vasculitis Therapy as Effective as Standard Care | National Institutes of Health (NIH)

  https://www.nih.gov/news-events/nih-research-matters/new-vasculitis-therapy-effective-standard-care

  Patients with severe vasculitis, or inflammation of the blood vessels, get the same benefits from just 4 doses of the drug rituximab over a month as from the standard daily therapy for 18 months, a new study reports. […] The current standard of care for ANCA-related vasculitis requires daily doses of the harsh immunosuppressant drug cyclophosphamide for 3 to 6 months. Daily doses of another immunosuppressant, azathioprine, then follow for a year or more. This standard therapy usually clears the vasculitis, but relapse is common. In addition, this treatment suppresses the immune system in a non-specific way and has potentially severe side effects. In contrast, the drug rituximab is specifically targeted to deplete the type of immune cells thought to produce ANCA. […] Short-term results, reported in 2010, found that 64% of those taking rituximab were free of disease after 6 months compared to 53% of those receiving standard care.

• #1 Rituximab : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/vasculitis-treatments/rituximab/

  Rituximab is a monoclonal antibody that is approved for the treatment of ANCA associated vasculitis. Rituximab is sometimes used to treat other forms of vasculitis, such as cryoglobulinemia and urticarial vasculitis. […] For initial treatment of vasculitis, Rituximab is usually given once per week for 4 weeks. Sometimes, it may instead be given as 2 infusions separated by 2 weeks. […] For maintenance treatment, Rituximab is usually given as one infusion every 6 months. Sometimes, the dosing interval can be lengthened to longer periods of time, such as 9 or 12 months.

• #1 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  Rituximab is an anti-CD20 monoclonal antibody that has been found to be effective for the treatment of many autoimmune disorders. […] Tocilizumab is a humanized recombinant anti-IL-6 receptor antibody that inhibits the binding of IL-6 to membrane-bound and soluble IL-6 receptors in a competitive manner. IL-6 supports the shift from acute to chronic inflammation and stimulates the release of ESR and CRP from hepatocytes. […] High-dose IVIG has a low incidence of severe side effects. More common side effects include nausea, headache, fatigue, and febrile infusion reactions. Rare but serious side effects include thrombosis and embolism, pulmonary edema, renal failure, aseptic meningitis, and severe anaphylactic reactions.

• #1 6 Vasculitis Medications To Help Manage the Disease

  https://health.clevelandclinic.org/vasculitis-medications

  Prednisone is a corticosteroid (glucocorticoid) that has proven effective in quickly treating inflammation from vasculitis. […] Rituximab is a DMARD approved for use in the treatment of two forms of vasculitis granulomatosis with polyangiitis (GPA or Wegeners) and microscopic polyangiitis (MPA). […] Avacopan is used to treat GPA and MPA, two types of anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. […] Cyclophosphamide is a tried-and-true DMARD used primarily for severe small- and medium-vessel vasculitis. […] Methotrexate is a DMARD used to treat vasculitis, as well as other autoimmune conditions. […] Doctors mainly use azathioprine as a maintenance medication in people with small- or medium-vessel vasculitis after the vasculitis has been managed. […] Though outcomes vary case by case, most people eventually see their vasculitis go into remission with medications and treatment. […] While we are unable to cure the condition, we can treat it and put it into remission while carefully watching for any potential side effects.

• #1 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  However, AAV continued to be associated with an elevated mortality risk. […] The introduction of cyclophosphamide in the 1960s was a breakthrough in changing the disease course when used in combination with existing steroid treatments. […] The advent of rituximab that there was a realistic prospect of reducing treatment-induced morbidity. […] Rituximab has indeed revolutionized the management of AAV and can achieve full drug-free remission in some patients, however, the treatment is not curative in the majority of cases; relapse rates are very high and even after successful rituximab maintenance treatment a return of B-cells and a rise in ANCA frequently occurs. […] In 2021 avacopan was approved for the treatment of AAV. […] Avacopan was non-inferior to prednisone at inducing clinical remission in patients with severe GPA or MPA on a background of rituximab or cyclophosphamide after 6 months in the ADVOCATE trial.

• #1 Treatment of Vasculitis | Hospital Clínic Barcelona

  https://www.clinicbarcelona.org/en/assistance/diseases/vasculitis/treatment

  The following biological agents have proven effective in certain types of vasculitis: Tumour necrosis factor (TNF) inhibitors such as infliximab, etanercept or adalimumab in the treatment of Takayasus arteritis. Anti-interleukin-6 (IL-6) agents such as tocilizumab for giant cell arteritis (GCA) and Takayasus arteritis. Anti-CD20 monoclonal antibodies (anti-B-cell) in ANCA-associated vasculitides such as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and cryoglobulinaemic vasculitis (particularly in association with hepatitis C virus infection). Anti-IL-5 agents such as mepolizumab in the case of eosinophilic granulomatosis with polyangiitis (EGPA). […] The long-term use of corticosteroids is associated with side effects such as weight loss, fat accumulation (Cushings syndrome), muscle atrophy and the development of osteoporosis, high blood pressure and glaucoma. Furthermore, some traditional immunosuppressants are related with a decreased blood cell count (e.g., leucocytes, red blood cells and platelets) and a minimal risk of developing cancer (above all in patients administered high doses over a long period). Corticosteroids and various immunosuppressants (traditional and biological) share the risk of producing infections because not only do they reduce the bodys defences to prevent it from being attacked by its own immune system, but they also reduce its ability to fight off the germs that cause infections.

• #1 General Vasculitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/general-vasculitis/

  Biologic agents such as rituximab, tocilizumab, and mepolizumab may be prescribed for specific types of vasculitis. Biologic medications are complex proteins derived from living organisms; they target certain parts of the immune system to control inflammation. […] For very severe cases, other additional treatments may include plasmapheresis (plasma exchange), intravenous gamma globulin, or surgery to restore blood flow.

• #1 Vasculitis – Treatment | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/vasculitis/treatment

  Immunomodulators lower the defense system activity (inflammation) that causes symptoms. Possible side effects can include gastrointestinal tract problems. […] Immunosuppressive medicines suppress or weaken the body’s ability to fight germs and sickness. Possible side effects include a higher risk of infection and birth defects. […] Interferon therapy blocks and reduces swelling. Interferons are molecules that the body’s defense system normally makes, but they have also been developed as medicines. […] Intravenous immunoglobulin (IVIG) helps control the body’s defensive response. This medicine also fights infection by introducing purified antibodies from healthy donors into the bloodstream. […] Monoclonal antibodies suppress the body’s natural defense system against illness. Possible side effects include fever-like symptoms, stomach pain, and allergic reactions. […] Tumor necrosis factor inhibitors suppress the body’s defense system by blocking a protein called tumor necrosis factor alpha.

• #1 Immunotherapy for Vasculitis | NYU Langone Health

  https://nyulangone.org/conditions/vasculitis/treatments/immunotherapy-for-vasculitis

  For more severe forms of vasculitis, such as granulomatosis with polyangiitis, your doctor may prescribe intravenous (IV) infusions of a protein called immunoglobulin. […] Intravenous immunoglobulin infusions are given daily for about five days over the course of several months on an outpatient basis. Most people receive regular maintenance therapy of IV immunoglobulin every few weeks after the initial round of treatment to keep the immune system suppressed until remission is achieved. […] You may again need IV immunoglobulin if your symptoms return, so its important that you follow up with your rheumatologist on a regular basis, so that you can be monitored for any signs of a flare up.

• #1 Types of Vasculitis Treatment

  https://www.uofmhealth.org/conditions-treatments/rheumatology/vasculitis-treatments

  Immunosuppressants used to treat vasculitis include: Cyclophosphamide, Rituximab, Methotrexate, Azathioprine, Mycophenolate mofetil, Leflunomide, Tocilizumab, TNF inhibitors (infliximab, adalimumab, certilizomab or golimumab). […] In some severe cases, a patient’s plasma, which contains harmful antibodies, may be exchanged for healthy plasma through a procedure similar to dialysis. […] When advanced forms of vasculitis cause significant damage to blood vessels, vascular bypass surgery may be performed to redirect the flow of blood around the damaged vessel. Surgery may also be required to repair or replace (via transplant) damaged or failed organs resulting from vasculitis. […] While getting clinical care at Michigan Medicine Vasculitis Program you might have the opportunity to participate in research and contribute to advancing our understanding and options for treatment for these rare diseases.

• #1 Treatment of ANCA Vasculitis | ANCA Vasculitis NewsEnvelope icon

  https://ancavasculitisnews.com/treatment-of-anca-vasculitis/

  ANCA vasculitis, or antineutrophil cytoplasmic antibody-associated vasculitis, is the name of a group of autoimmune conditions characterized by the inflammation of the blood vessels caused by the immune system mistakenly attacking them. […] There is currently no cure for ANCA vasculitis, but there are treatments available to manage the condition. Current treatments aim to push the condition into remission, where no symptoms appear for a time, and to maintain remission. Depending on the severity and type of ANCA vasculitis, different therapies may be used. […] Glucocorticoids, such as prednisolone, act as an anti-inflammatory. By reducing inflammation, they can reduce the damage caused by the immune system. These are the most commonly used treatments for ANCA vasculitis and are often used in combination with other therapies to induce and maintain remission.

• #1 Novel Insights into ANCA-associated Vasculitis Treatment Market

  https://www.delveinsight.com/blog/anca-associated-vasculitis-treatment

  The ANCA-associated vasculitis treatment begins with remission induction, which is typically accomplished with cyclophosphamide, rituximab, and high-dose steroids. Plasma exchange may be utilized in conjunction with induction treatment in cases of life-threatening illness or significant renal involvement. Methotrexate or azathioprine is frequently used to keep the patient in remission. Although there is no agreement on the length of maintenance, it is often administered for 18-24 months to prevent recurrence. In addition, rituximab is suggested to keep GPA and MPA patients in remission. […] Developing novel ANCA-associated vasculitis treatment options towards an all-inclusive treatment that is devoid of related toxicities and a glucocorticoid-free regimen is required for ANCA-associated vasculitis treatment. Emerging therapies may fill such gaps in the future.

• #1 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  The latest EULAR treatment recommendations for maintenance treatment favor rituximab over azathioprine due to its superior success record in relapse prevention. […] The current EULAR guidelines highlight the reduction of glucocorticoid exposure to improve patient wellbeing as a key goal in the management of AAV, and several of the latest recommendations are focused on achieving this. […] Evaluating success and optimizing outcomes in the management of AAV thus requires routine monitoring using a combination of assessments to evaluate the extent of AAV damage, susceptibility to comorbidities, and the impact of AAV symptoms and adverse events on the patient as well as AAV disease activity, and treatment strategy modified accordingly.

• #1 Vasculitis Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/vasculitis.html

  Treatment varies by type of vasculitis: […] Polyarteritis nodosa– This form of vasculitis usually is treated with prednisone (sold under several brand names) and, in most cases, an additional immunosuppressant such as cyclophosphamide (Cytoxan, Neosar), azathioprine (Imuran), methotrexate (Rheumatrex, others), mycophenolate (CellCept), or rituximab (Rituxan). […] Cutaneous leukocytoclastic angiitis – This form often goes away on its own without treatment. Any newly started medication that may have triggered the vasculitis should be stopped, if possible. For severe cases, corticosteroids may be given. […] Giant cell arteritis– Treatment begins with high doses of prednisone, which gradually are reduced over many months. If symptoms return, steroid treatment may be necessary for a year or more. Additional medications, such as tocilizumab (Actemra) or methotrexate (Rheumatrex and others), may be recommended.

• #1 Vasculitis Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/vasculitis.html

  Granulomatosis with polyangiitis– Standard treatment includes prednisone along with cyclophosphamide or rituximab. Methotrexate (Rheumatrex and others), azathioprine (Imuran), mycophenolate (CellCept), or other immune-suppressing medications may be used for maintenance therapy or when initial treatments are not effective. In some severe cases, a procedure called plasma exchange may be recommended. With plasma exchange, blood is taken out of the patient, the liquid portion of the blood (called plasma) is removed, and the blood cells along with plasma from a blood donor or a plasma substitute are transfused back into the person. […] Takayasu’s arteritis– Corticosteroids are used to treat this condition. However, other immune-suppressing medications may be recommended if improvement is not adequate with corticosteroids alone. Narrowed arteries may need to be corrected with surgery or angioplasty (inserting a balloon-tipped catheter to widen the vessels).

• #1 Vasculitis and Thrombophlebitis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1008239-treatment

  Some patients respond to NSAIDs alone; however, most require treatment with prednisone. Steroid-sparing agents may be needed (eg, methotrexate, mycophenolate mofetil, colchicine, IVIG). Penicillin prophylaxis may prevent disease exacerbations in patients with evidence of triggering streptococcal infections. […] […] Induction therapy usually involves high-dose glucocorticoid (prednisone, 1 mg/kg/d). The initial high dose should be maintained for a month and then gradually tapered. Azathioprine or methotrexate have been used as adjuncts to steroid therapy in patients with Takayasu arteritis to improve disease control and to facilitate reduction of the steroid dose. Cyclophosphamide has been used in adults with Takayasu arteritis resistant to glucocorticoids. In addition, tumor necrosis factor (TNF)- inhibitors (eg, infliximab, etanercept) have been tried with encouraging results, including in a small study of 4 children. […] […] Anticoagulation is indicated for any patient with a thrombotic episode and an underlying hypercoagulable state. This usually involves initial treatment with heparin with subsequent transition to warfarin.

• #1 Vasculitis and Thrombophlebitis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1008239-treatment

  The management of children with Kawasaki disease involves hospital admission and treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin in the acute phase of the illness. Subsequently, daily low-dose aspirin is given for 6-8 weeks until follow-up echocardiography. IVIG-resistant disease may be treated with methylprednisolone and/or other immunosuppressive therapies (see Kawasaki Disease Treatment of IVIG-Resistant Disease). Guidelines from the American College of Rheumatology and the Vasculitis Foundation conditionally recommend the use of adjunctive glucocorticoids with IVIG as initial therapy for patients with acute Kawasaki disease who are at high risk for IVIG resistance or the development of coronary artery aneurysms. […] […] The management of children with Henoch-Schnlein purpura is primarily symptomatic, and most patients do not require hospital admission. Nonsteroidal anti-inflammatory drugs (NSAIDs) can be given for joint pain or swelling. Corticosteroids may be considered in selected patients (ie, those with severe GI symptoms), but is an area of controversy in the literature. Clinically significant nephritis is typically treated with steroids and other immunosuppressive therapies. […]

• #1 Vasculitis: Symptoms, Types & Treatment

  https://my.clevelandclinic.org/health/diseases/12101-vasculitis

  Most people can manage their symptoms with medication. […] Most people with vasculitis can manage their symptoms with medication. But vasculitis can be fatal if you experience severe symptoms that affect blood flow to your organs. […] Your provider will suggest treatments that manage the symptoms youre experiencing. Theyll help you find treatments that prevent damage to your blood vessels and organs. Some medications your provider might prescribe include: Corticosteroids, Immunosuppressants or biologics, ACE inhibitors. […] Theres no cure for vasculitis. Once you find treatments that manage your symptoms well, you might enter remission long periods of time between episodes of symptoms. Some people are in remission for months or years before experiencing symptoms again.

• #1 Leukocytoclastic Vasculitis Treatment: Options & Monitoring | Maggie Yu MD, IFMCP

  https://drmaggieyu.com/blog/leukocytoclastic-vasculitis-treatment-options-monitoring/

  Steroid-sparing agents may be used for chronic or resistant cases of leukocytoclastic vasculitis. […] Immunosuppressive therapy is used when there is significant cutaneous involvement, vasculitis, or systemic symptoms. […] Identifying and treating underlying conditions, such as vasculitis, is crucial for effective management. […] Regular monitoring helps assess treatment effectiveness and detect complications early, particularly in cases of vasculitis. […] Regular follow-ups are crucial for patients with leukocytoclastic vasculitis. […] Long-term corticosteroid or immunosuppressive therapy can have significant side effects, including vasculitis. […] In severe cases, leukocytoclastic vasculitis can affect internal organs. […] Chronic leukocytoclastic vasculitis requires ongoing treatment.

• #1 Progress in treatment of ANCA-associated vasculitis | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar3797

  Autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis (AAV) is characterised by inflammation of blood vessels. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide transformed AAV from a fatal condition to a largely treatable condition. […] There is, however, a high unmet need in the treatment of AAV. A proportion of patients are refractory to current therapies; 50% experience a relapse within 5 years and treatment toxicity contributes to mortality and chronic disability. […] As knowledge of the pathogenesis of vasculitis grows, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of vasculitis. Lymphocyte-targeted and cytokine-targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes.

• #1

  https://journals.lww.com/cjasn/fulltext/2021/12000/what_is_the_best_maintenance_therapy_for_anca.24.aspx

  For patients who do not have access to or are allergic to rituximab, maintenance treatment with azathioprine remains the preferred choice. […] For patients at high risk of relapse, a longer (4-year) duration of maintenance therapy seems to be associated with improved relapse-free survival. […] The choice of maintenance therapy should be individualized on the basis of an assessment of the risk of relapse, the effect that a relapse would have (e.g., because of poor pulmonary or kidney reserve), the risks and costs of immunosuppression, and the patients preference.

• #1 Maintenance therapy in well-controlled vasculitis: Is it ever ok to stop? • Johns Hopkins Rheumatology

  https://www.hopkinsrheumatology.org/2018/07/maintenance-therapy-in-well-controlled-vasculitis-is-it-ever-ok-to-stop/

  Whether or not and how to taper off the last bit of medication for patients with clinically stable ANCA-associated vasculitis are hotly debated topics in the vasculitis field. […] Currently, people with vasculitis are treated with strong medications that inhibit the immune system (immunosuppressive drugs) and help them stay in a disease-free state. […] While most patients received maintenance therapy with low-dose steroids for some time after withdraw of immunosuppressive drugs, 3 did not and 15 were weaned completely off of steroids during the follow up period. […] This work strengthens the emerging view that select patients with ANCA-associated vasculitis may be able to come off immunosuppression completely and remain in remission.

• #1 Overview of Vasculitis – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/overview-of-vasculitis

  All patients treated with immunosuppressants should be monitored for opportunistic and other infections. Testing for tuberculosis, hepatitis B, and hepatitis C, which can become reactivated or exacerbated by some immunosuppressive therapies, should be done. Prophylaxis against should be considered for patients receiving potent or prolonged immunosuppressive therapy. […] In most forms of vasculitis, initial treatment begins with corticosteroids, and an additional immunosuppressant. Treatment approach depends on disease severity. […] Severe, rapidly progressive and life- or organ-threatening vasculitis (eg, causing alveolar hemorrhage, rapidly progressive glomerulonephritis, or mesenteric ischemia) is a medical emergency requiring hospital admission and immediate treatment. High-dose corticosteroids are typically prescribed.

• #1 Vasculitis | Symptoms, causes | Versus Arthritis

  https://versusarthritis.org/about-arthritis/conditions/vasculitis/

  In some types of vasculitis (such as granulomatosis with polyangiitis), an infection may trigger a relapse. You may therefore be given antibacterial drugs such as co-trimoxazole to protect against this. These drugs can also help to protect against the increased risk of infection caused by the stronger immunosuppressive drugs. […] Cyclophosphamide, for example, can cause bleeding from the bladder, hair thinning and an increased risk of infection. Unfortunately, there’s also a significant risk that it can reduce fertility in both men and women. […] Because of these risks, cyclophosphamide will be stopped or exchanged for a different immunosuppressive drug as soon as your vasculitis is controlled. This is usually azathioprine, but methotrexate or mycopheonlate might be used instead. Rituximab, a biological therapy given by intravenous infusion, can also help to encourage remission in some types of vasculitis.

• #1 Vasculitis | healthdirect

  https://www.healthdirect.gov.au/vasculitis

  You may need to take medicines for vasculitis for a long time, and they may have side effects, including your ability to fight infection. Talk to your doctor about influenza, pneumonia, and shingles vaccines to reduce your risk. […] It is also important to talk to your doctor about how to reduce the side effects of your medicines.

• #1 Overview of the management of vasculitis in adults – UpToDate

  https://www.uptodate.com/contents/overview-of-the-management-of-vasculitis-in-adults

  Overview of the management of vasculitis in adults […] These conditions are often serious and sometimes fatal diseases that require prompt recognition and therapy. […] Treatment recommendations for specific forms of vasculitis are reviewed for each disorder separately […] The framework for the treatment of vasculitis is similar to that used for many other systemic autoimmune rheumatic disorders, while the specific therapeutic regimen depends upon the nature and severity of the particular disorder. […] The management scheme for vasculitis generally includes the following components: […] Remission induction – The goal of initial treatment is to induce remission of the disease. Initial management usually involves medium to high doses of glucocorticoids, with the addition of an immunosuppressive agent in most forms of disease. […] However, a major goal of recent clinical trials and introduction of new treatments for vasculitis is to develop regimens supported by good data that allows for substantially reducing or eliminating the doses of glucocorticoids used for induction of remission („glucocorticoid-sparing”).

• #1 How We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy

  https://www.mdpi.com/2077-0383/14/1/208

  Despite these advancements, the response to cessation of treatment varies widely among patients. […] The effective stratification of patients into low or high-risk categories for relapse is feasible using several factors. […] This approach promises a more nuanced and effective management of AAV, potentially reducing unnecessary exposure to long-term immunosuppression while maintaining disease control.

• #1 Dr. Patrick Nachman & Treating ANCA Vasculitis with Rituximab | Department of Medicine

  https://www.med.unc.edu/medicine/news/chairs-corner/podcast/treating-with-rituximab/

  The other possibility is thinking about introducing plasmapheresis again to remove the offending ANCA antibodies from the circulation. […] By far, the biggest concern is the risk of infections. […] Rituximab as the infusion is an expensive medication, which may be different depending upon insurance plans, but there is a real out-of-pocket cost for the co-pay for this biological drug. […] Azathioprine is a medication that is taken daily by mouth, and the immense majority of patients have few adverse events, if any. […] What our approach has been over the years, is if the patient has done really well and has absolutely no signs or symptoms suggestive of active disease after a semi-arbitrary period of time of a year or so, then we try to follow the patient by gradually minimizing immunosuppression and getting them off immunosuppression. […] There are different immunosuppressants that are being explored.

• #1 Small-Vessel Vasculitis (Leukocytoclastic Vasculitis) Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333891-treatment

  Second-line treatments include traditional steroid-sparing agents, especially for patients with severe disease that flares with taper of systemic glucocorticoids: Methotrexate, 10-25 mg weekly; however, this agent has been implicated as a trigger of vasculitis in some cases; Azathioprine, 2 mg/kg daily; Mycophenolate, 2-3 g daily. […] Adjunctive therapies, or as monotherapy in select patients, may include: Hydroxychloroquine 200-400 mg daily (particularly for urticarial vasculitis); Rituximab (generally reserved for ANCA-associated vasculitis and cryoglobulinemic vasculitis); Intravenous immunoglobulin; Janus kinase (JAK) inhibitors may have a role to play in the treatment of cutaneous vasculitis, and provide an exciting new potential therapy for refractory disease. […] It should be noted that data for all therapies are very limited. Currently, an international, multicenter, randomized trial enrolling patients with isolated skin vasculitis (ARAMIS; NCT02939573) is investigating colchicine, dapsone, and azathioprine as first-line therapies. The only completed randomized controlled trial in cutaneous vasculitis suggested that colchicine had no significant therapeutic effect. However, this study was limited by short treatment duration (1 month) and relapse upon cessation of the medication in 3 complete responders (suggesting the medication was helpful in a subset of the patients studied). […] Additional medications that have been reported in isolated patients with cutaneous small-vessel vasculitis include infliximab and cyclosporine.

• #1 How We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy

  https://www.mdpi.com/2077-0383/14/1/208

  Recent studies, both retrospective and prospective, have further explored the efficacy of repeated RTX doses for maintenance therapy. […] The ideal frequency and dosage of RTX infusions, as well as the optimal duration for maintenance therapy, remain unresolved. […] Our approach to maintenance therapy once remission is achieved is schematized in Figure 1. […] Our findings suggest that for MPO-associated ANCA vasculitis, ongoing maintenance therapy may not be necessary after an effective B-cell depletion induction. […] The experience at G. Bosco Hospital supports the notion that an intensive protocol for B-cell depletion during remission induction, without routine subsequent immunosuppression, could offer a more favorable approach compared to fixed dosing of RTX, particularly in light of the delayed relapse observed in many cases.

• #1 Treatment of Vasculitis: Beyond the Basics | IntechOpen

  https://www.intechopen.com/chapters/72404

  Tocilizumab works against the pro-inflammatory cytokine Interleukin 6 and has proven its efficacy in Takayasu arteritis that has failed to respond to Infliximab. Giant cell arteritis, non responsive to various other biological agents, has reacted remarkably to Tocilizumab by achieving disease remission in most of the cases. Despite all its applauding outcomes, life-threatening infection remains a serious complication. […] In recent times use of high dose corticosteroids, cytotoxic and immunosuppressant drugs has improved the prognosis of systemic vasculitis dramatically. However, some patients still do not respond to conventional therapy or may not achieve remission. Few of them would relapse and a large number of patients develop illness secondary to the adverse effects caused by long term use of these drugs. The advent of biological agents has not just let to a better understanding of pathophysiology of systemic vasculitis, but has also proved to be safe and efficacious. Among these agents, anti-TNF and anti-B cell therapy have been the first choice in many cases. Although clinical data are still insufficient, these agents seem to occupy most of the market in near future.

• #1 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  Health-related quality of life is a key contributor to overall well-being, and this is becoming an increasingly prominent factor when making therapeutic choices in the management of ANCA-associated vasculitis (AAV). […] Progress in available therapeutic strategies for AAV has resulted in this historically acute disease with a potentially fatal short-term outcome, becoming a relapsing-remitting chronic disorder. […] This new perspective on AAV means that patient survival should no longer be considered as the only major treatment target. Additional outcomes in this context that should be portrayed in order to consider a therapeutic approach as successful include patient quality of life, as well as the burden of treatment-induced morbidity. […] Ongoing monitoring of comorbidity risk and of quality of life is thus key for successful AAV management.

• #1 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  With these shifts in treatment capabilities and understanding of patient burden, it is necessary to adjust the treatment paradigm accordingly. Treatment success is no longer defined solely by the control of disease activity; treatment success requires holistic improvement determined through the assessment of all aspects of the disease, ranging from disease control to comorbidity risk through to the assessment of health-related quality of life. […] The management of AAV has entered a new era with a strong focus on both the management and prevention of comorbidities as well as patient-reported outcomes, both of which are now considered key factors in defining treatment success. […] Immunosuppressive and anti-inflammatory treatments, mainly cyclophosphamide and prednisolone, have helped control the progression of AAV and increased the mean 5-year survival rate for patients with AAV.

• #2 Overview of the management of vasculitis in adults – UpToDate

  https://www.uptodate.com/contents/overview-of-the-management-of-vasculitis-in-adults

  Overview of the management of vasculitis in adults […] These conditions are often serious and sometimes fatal diseases that require prompt recognition and therapy. […] Treatment recommendations for specific forms of vasculitis are reviewed for each disorder separately […] The framework for the treatment of vasculitis is similar to that used for many other systemic autoimmune rheumatic disorders, while the specific therapeutic regimen depends upon the nature and severity of the particular disorder. […] The management scheme for vasculitis generally includes the following components: […] Remission induction – The goal of initial treatment is to induce remission of the disease. Initial management usually involves medium to high doses of glucocorticoids, with the addition of an immunosuppressive agent in most forms of disease. […] However, a major goal of recent clinical trials and introduction of new treatments for vasculitis is to develop regimens supported by good data that allows for substantially reducing or eliminating the doses of glucocorticoids used for induction of remission („glucocorticoid-sparing”).

• #2 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  In 1954, criteria for diagnosing Wegeners granulomatosis (WG) was published. Corticosteroids, cyclophosphamide, and trimethoprim/sulfamethoxazole were later recommended for the treatment of Wegeners granulomatosis. […] Glucocorticoids are the cornerstone of vasculitis treatment. Oral prednisone is the drug and route of administration of choice, while IV pulse methylprednisolone is an alternative option for the treatment of GCA, Takayasu arteritis, and AAV. High-dose oral glucocorticoids is often combined with non-glucocorticoids immunosuppressive therapy to induce remission in large vessel vasculitis. […] Corticoid-sparing immunosuppressants include azathioprine, methotrexate, mycophenolate mofetil, and cyclophosphamide. These agents are used in large, medium, and small vessel vasculitis. It is also given in cases of refractoriness to prednisone and other drugs.

• #2 Progress in treatment of ANCA-associated vasculitis | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar3797

  Treatment-related toxicity, together with the 10 to 30% of patients who do not respond to traditional immunosuppressive agents and pursue a refractory course and the 50% of patients who relapse within 5 years despite continued immunosuppression, has shifted the focus of treatment for AAV. […] From learning how to best use traditional agents to minimise toxicity without losing efficacy, to the advent of new targeted biological agents, a second revolution in the treatment of AAV is on the horizon. […] After making a diagnosis of systemic vasculitis, treatment is divided into remission induction, then 3 to 6 months of intensive therapy, followed by a longer period of remission maintenance therapy. […] Current regimens start oral prednisone/prednisolone at 1 mg/kg/day, reducing to 10 to 20 mg daily by 12 weeks, with up to 3 g intravenous (i.v.) methylprednisolone for generalised or severe presentations.

• #2 Overview of Vasculitis – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/overview-of-vasculitis

  All patients treated with immunosuppressants should be monitored for opportunistic and other infections. Testing for tuberculosis, hepatitis B, and hepatitis C, which can become reactivated or exacerbated by some immunosuppressive therapies, should be done. Prophylaxis against should be considered for patients receiving potent or prolonged immunosuppressive therapy. […] In most forms of vasculitis, initial treatment begins with corticosteroids, and an additional immunosuppressant. Treatment approach depends on disease severity. […] Severe, rapidly progressive and life- or organ-threatening vasculitis (eg, causing alveolar hemorrhage, rapidly progressive glomerulonephritis, or mesenteric ischemia) is a medical emergency requiring hospital admission and immediate treatment. High-dose corticosteroids are typically prescribed.

• #2 Types of Vasculitis Treatment

  https://www.uofmhealth.org/conditions-treatments/rheumatology/vasculitis-treatments

  The key to lessening the symptoms of the many types of vasculitis is to reduce inflammation. Anti-inflammatory medications, notably glucocorticoids such as prednisone or methylprednisolone, are the most common first-line treatments. Glucocorticoids are effective, but patients who take them over a long time period may experience side effects and toxicities. […] Medications that suppress the body’s immune system, such as rituximab or cyclophosphamide, may also be used to reduce dosages of glucocorticoids and reduce the long-term risks associated with them. […] In general, the approach is to use a strong drug (for 3 to 6 months) to put the vasculitis in remission (where there is no sign of active disease) and then a more mild drug (over 12 to 36 months or longer) to maintain that remission and protect from flare ups of disease.

• #2 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  Cyclophosphamide is an alkylating agent that blocks DNA replication. Low doses of cyclophosphamide with concomitant glucocorticoids are used in the treatment of vasculitis. A combination of high-dose glucocorticoid and cyclophosphamide improved survival rates from 20% to over 80% in patients with AAV, making this combination the standard treatment. […] Methotrexate inhibits dihydrofolate reductase and activation of nuclear factor-B. It is used to treat various large, medium, and small vessel vasculitis as well as Behet disease. Methotrexate or azathioprine are either first or second line options for remission maintenance in patients with AAV. […] TNF inhibitors approved by the US Food and Drug Administration (FDA) and utilized for vasculitis treatment include infliximab, adalimumab, and etanercept.

• #2 Vasculitis | Symptoms, causes | Versus Arthritis

  https://versusarthritis.org/about-arthritis/conditions/vasculitis/

  In some types of vasculitis (such as granulomatosis with polyangiitis), an infection may trigger a relapse. You may therefore be given antibacterial drugs such as co-trimoxazole to protect against this. These drugs can also help to protect against the increased risk of infection caused by the stronger immunosuppressive drugs. […] Cyclophosphamide, for example, can cause bleeding from the bladder, hair thinning and an increased risk of infection. Unfortunately, there’s also a significant risk that it can reduce fertility in both men and women. […] Because of these risks, cyclophosphamide will be stopped or exchanged for a different immunosuppressive drug as soon as your vasculitis is controlled. This is usually azathioprine, but methotrexate or mycopheonlate might be used instead. Rituximab, a biological therapy given by intravenous infusion, can also help to encourage remission in some types of vasculitis.

• #2

  https://link.springer.com/article/10.1007/s40674-016-0045-8

  Rituximab, a chimeric monoclonal antibody, depletes B cells by ligation with surface-expressed CD20 antigens. […] These two trials had some differences. […] In both trials, rituximab was administered as four infusions of 375 mg/m2 body surface area; however, in RITUXVAS, two or three cycles of cyclophosphamide were given in addition to rituximab. […] Methotrexate at a dose of 15 to 25 mg per week (oral or subcutaneously) is used as an alternative to cyclophosphamide and rituximab therapy in patients with early systemic disease without significant renal involvement. […] Mycophenolate mofetil (MMF) is used routinely in systemic lupus erythematosus as an induction agent, and evidence from a retrospective case series and a prospective pilot trial suggested benefit in AAV as well. […] There is little direct evidence to guide glucocorticoid dosing, despite their use in induction therapy for many years.

• #2 Small-Vessel Vasculitis (Leukocytoclastic Vasculitis) Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333891-treatment

  Second-line treatments include traditional steroid-sparing agents, especially for patients with severe disease that flares with taper of systemic glucocorticoids: Methotrexate, 10-25 mg weekly; however, this agent has been implicated as a trigger of vasculitis in some cases; Azathioprine, 2 mg/kg daily; Mycophenolate, 2-3 g daily. […] Adjunctive therapies, or as monotherapy in select patients, may include: Hydroxychloroquine 200-400 mg daily (particularly for urticarial vasculitis); Rituximab (generally reserved for ANCA-associated vasculitis and cryoglobulinemic vasculitis); Intravenous immunoglobulin; Janus kinase (JAK) inhibitors may have a role to play in the treatment of cutaneous vasculitis, and provide an exciting new potential therapy for refractory disease. […] It should be noted that data for all therapies are very limited. Currently, an international, multicenter, randomized trial enrolling patients with isolated skin vasculitis (ARAMIS; NCT02939573) is investigating colchicine, dapsone, and azathioprine as first-line therapies. The only completed randomized controlled trial in cutaneous vasculitis suggested that colchicine had no significant therapeutic effect. However, this study was limited by short treatment duration (1 month) and relapse upon cessation of the medication in 3 complete responders (suggesting the medication was helpful in a subset of the patients studied). […] Additional medications that have been reported in isolated patients with cutaneous small-vessel vasculitis include infliximab and cyclosporine.

• #2 Progress in treatment of ANCA-associated vasculitis | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar3797

  Autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis (AAV) is characterised by inflammation of blood vessels. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide transformed AAV from a fatal condition to a largely treatable condition. […] There is, however, a high unmet need in the treatment of AAV. A proportion of patients are refractory to current therapies; 50% experience a relapse within 5 years and treatment toxicity contributes to mortality and chronic disability. […] As knowledge of the pathogenesis of vasculitis grows, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of vasculitis. Lymphocyte-targeted and cytokine-targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes.

• #2 Rituximab : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/vasculitis-treatments/rituximab/

  Rituximab is a monoclonal antibody that is approved for the treatment of ANCA associated vasculitis. Rituximab is sometimes used to treat other forms of vasculitis, such as cryoglobulinemia and urticarial vasculitis. […] For initial treatment of vasculitis, Rituximab is usually given once per week for 4 weeks. Sometimes, it may instead be given as 2 infusions separated by 2 weeks. […] For maintenance treatment, Rituximab is usually given as one infusion every 6 months. Sometimes, the dosing interval can be lengthened to longer periods of time, such as 9 or 12 months.

• #2 New ANCA-Associated Vasculitis treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=101

  Cyclophosphamide, a potent immunosuppressive agent, is highly effective in inducing remission, especially in severe cases involving kidney or lung damage. […] Rituximab, a monoclonal antibody targeting B-cells, is FDA-approved for induction and maintenance therapy in AAV and offers similar efficacy with potentially fewer long-term side effects. […] Azathioprine and methotrexate are commonly used as maintenance therapies following induction with cyclophosphamide or rituximab. […] Tavneos (avacopan) is a newly FDA-approved medication specifically indicated for ANCA-associated vasculitis. […] Rituximab is a monoclonal antibody that targets CD20-positive B cells, reducing antibody production and inflammation. […] Cyclophosphamide is a potent immunosuppressive agent traditionally used for severe AAV cases, particularly those involving major organs such as kidneys or lungs.

• #2 Treatment of Vasculitis: Beyond the Basics | IntechOpen

  https://www.intechopen.com/chapters/72404

  Tocilizumab works against the pro-inflammatory cytokine Interleukin 6 and has proven its efficacy in Takayasu arteritis that has failed to respond to Infliximab. Giant cell arteritis, non responsive to various other biological agents, has reacted remarkably to Tocilizumab by achieving disease remission in most of the cases. Despite all its applauding outcomes, life-threatening infection remains a serious complication. […] In recent times use of high dose corticosteroids, cytotoxic and immunosuppressant drugs has improved the prognosis of systemic vasculitis dramatically. However, some patients still do not respond to conventional therapy or may not achieve remission. Few of them would relapse and a large number of patients develop illness secondary to the adverse effects caused by long term use of these drugs. The advent of biological agents has not just let to a better understanding of pathophysiology of systemic vasculitis, but has also proved to be safe and efficacious. Among these agents, anti-TNF and anti-B cell therapy have been the first choice in many cases. Although clinical data are still insufficient, these agents seem to occupy most of the market in near future.

• #2 Treatment of Vasculitis | Hospital Clínic Barcelona

  https://www.clinicbarcelona.org/en/assistance/diseases/vasculitis/treatment

  The following biological agents have proven effective in certain types of vasculitis: Tumour necrosis factor (TNF) inhibitors such as infliximab, etanercept or adalimumab in the treatment of Takayasus arteritis. Anti-interleukin-6 (IL-6) agents such as tocilizumab for giant cell arteritis (GCA) and Takayasus arteritis. Anti-CD20 monoclonal antibodies (anti-B-cell) in ANCA-associated vasculitides such as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and cryoglobulinaemic vasculitis (particularly in association with hepatitis C virus infection). Anti-IL-5 agents such as mepolizumab in the case of eosinophilic granulomatosis with polyangiitis (EGPA). […] The long-term use of corticosteroids is associated with side effects such as weight loss, fat accumulation (Cushings syndrome), muscle atrophy and the development of osteoporosis, high blood pressure and glaucoma. Furthermore, some traditional immunosuppressants are related with a decreased blood cell count (e.g., leucocytes, red blood cells and platelets) and a minimal risk of developing cancer (above all in patients administered high doses over a long period). Corticosteroids and various immunosuppressants (traditional and biological) share the risk of producing infections because not only do they reduce the bodys defences to prevent it from being attacked by its own immune system, but they also reduce its ability to fight off the germs that cause infections.

• #2 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  However, AAV continued to be associated with an elevated mortality risk. […] The introduction of cyclophosphamide in the 1960s was a breakthrough in changing the disease course when used in combination with existing steroid treatments. […] The advent of rituximab that there was a realistic prospect of reducing treatment-induced morbidity. […] Rituximab has indeed revolutionized the management of AAV and can achieve full drug-free remission in some patients, however, the treatment is not curative in the majority of cases; relapse rates are very high and even after successful rituximab maintenance treatment a return of B-cells and a rise in ANCA frequently occurs. […] In 2021 avacopan was approved for the treatment of AAV. […] Avacopan was non-inferior to prednisone at inducing clinical remission in patients with severe GPA or MPA on a background of rituximab or cyclophosphamide after 6 months in the ADVOCATE trial.

• #2 Vasculitis | Symptoms, causes | Versus Arthritis

  https://versusarthritis.org/about-arthritis/conditions/vasculitis/

  If you have vasculitis affecting small and/or medium-sized blood vessels, then you may only need a small dose of steroids to control it. […] However, you may need a combination of steroids and immunosuppressive drugs, probably over several years, especially if vasculitis affects internal organs. For many types of vasculitis, including those affecting the kidney, lungs or other vital organs (especially if it involves both small and medium-sized blood vessels), your treatment will be given in stages. […] Plasma exchange (also known as blood washing) involves being connected to a machine that your blood passes through before being returned to you so it can be cleaned of the factors causing the vasculitis. Only a few people with the most severe types of vasculitis for example who have very severe kidney or lung disease will need plasma exchange. This will be done in specialist centres.

• #2 Understanding Your Vasculitis Treatment Options | Rheumatology located in Tustin and Laguna Hills, CA | Pacific Rheumatology Center

  https://www.pacificrheumatologycenter.com/post/understanding-your-vasculitis-treatment-options

  Since many types of vasculitis are caused by an overactive immune system, you may need medications that inhibit or prevent immune activity. […] In addition to reducing inflammation, corticosteroids also help suppress your immune system. However, we may prescribe other immunosuppressants, such as methotrexate, azathioprine, and cyclophosphamide. […] Several biologics are available to treat severe cases of certain types of vasculitis. If you need this treatment, your biologic medication is given through an intravenous (IV) infusion or injection because the proteins would be destroyed in your digestive tract. […] IVIG infusions are used to treat people with antibody deficiencies. We also prescribe IVIG infusions to reduce inflammation and stop the immune system from attacking your blood vessels.

• #2 Vasculitis: Causes, Symptoms, and Treatments

  https://www.webmd.com/rheumatoid-arthritis/vasculitis-treatment

  Sometimes vasculitis can cause issues that need surgery to repair. For instance, if your blood vessel walls bulge and form an aneurysm, surgery can lower the chances that it will burst. If you have a blocked artery, you could need surgery to restore blood flow to the area. But any kind of organ damage might require surgery.

• #2 Vasculitis Types & Symptoms | The University of Kansas Health System

  https://www.kansashealthsystem.com/care/conditions/vasculitis

  Vasculitis treatment is an oral corticosteroid, which may be recommended for long-term therapy. […] In some cases, surgery may be necessary to repair the damage caused by vasculitis for example, to repair an aneurysm. Arteries that are blocked by the effects of vasculitis can require surgery to restore the flow of blood to the affected organ.

• #2 Vasculitis Treatment and Care | Articularis Specialists

  https://www.arshealthcare.com/vasculitis

  Glucocorticoids (Deltasone) are used to treat inflammation in many types of vasculitis. […] For milder vasculitis, methotrexate (Rheumatrex), azathioprine (Imuran) and other immunosuppressants may be used. […] Newer biologic drugs can treat certain types of vasculitis, such as rituximab (Rituxan) for GPA, MPA; tocilizumab (Actemra) for GCA; and mepolizumab (Nucala) for EGPA. […] Cyclophosphamide (Cytoxan) is an immunosuppressant used for life threatening disease. […] Some patients with severe disease receive treatments like plasma exchange (plasmapharesis) or intravenous immunoglobulin (IVIG). […] If severe vasculitis damages blood vessels or organs, surgery may be needed to repair them. This may include vascular bypass grafting, sinus surgery or kidney transplant.

• #2 ANCA Vasculitis | UNC Kidney Center

  https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/anca-vasculitis/

  ANCA vasculitis is an autoimmune disease affecting small blood vessels in the body. […] Treatment of ANCA vasculitis is complicated and should include specialists for the organs/body parts involved. […] Because the immune system (antibodies and white blood cells) is causing the inflammation and damage, treatment involves trying to suppress or quiet the immune system. […] In general, treatment for ANCA vasculitis involves 2 parts. The first part is called induction therapy, which is aimed at trying to get the disease controlled and into remission. Once the disease goes into remission, the second part of treatment is called maintenance therapy. Maintenance therapy involves continuing immunosuppressive medications to keep the disease in remission and decrease the chance that it will come back (relapse).

• #2 Vasculitis Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/vasculitis.html

  Kawasaki disease– Treatment includes aspirin and a drug called gamma globulin given in high doses intravenously (into a vein) to reduce the risk of coronary artery damage. In some cases, corticosteroid therapy is recommended as well. […] There are a number of other types of vasculitis; they tend to be treated with immune-suppressing medications as described above.

• #2 HealthtalkTreating systemic vasculitis | Real People. Real life experiences. | Healthtalk

  https://healthtalk.org/experiences/systemic-vasculitis/treating-systemic-vasculitis/

  Vasculitis treatment involves drugs which suppress the immune system to stop the body attacking its own blood vessels. These drugs all have side effects. People told us it was routine to start with “aggressive” high dose steroids (prednisolone), either alone or together with other medication. […] For some types of vasculitis, people also had a type of chemotherapy (cyclophosphamide). […] When people were very unwell with vasculitis, they sometimes had other treatments such as blood transfusions, plasma exchange (to remove plasma from the blood and replace it with new plasma) and kidney dialysis. Treatment decisions often had to be made quickly and – because they were unwell – people were not always aware of what was happening at the time. […] To keep vasculitis under control, people had maintenance treatment. This could include a lower dose of steroids, other standard immunosuppressants, and biologic immunosuppressants. For some people it also included medication for high blood pressure, pain and infections. […] People varied in their response to immunosuppression. We heard many examples of how people enjoyed a better quality of life once vasculitis was treated. We also heard that they could be left with some symptoms as well as the damage that had already been done.

• #2 Treating Vasculitis – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/treating-vasculitis

  There is no cure for many of the vasculitis diseases. The aim of treatment is to keep the disease process in remission. […] Doctors treating patients with vasculitis rely on clinical trials and research being conducted and published to know what the best treatments are for different types of vasculitis. […] Unfortunately most types of vasculitis are rare and this makes it very difficult to do useful research and conduct clinical trials to find the best treatments. […] If you are being treated in a clinic that is involved in vasculitis research and clinical trials you may be invited to take part.

• #2 Progress in treatment of ANCA-associated vasculitis | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar3797

  For those presenting with severe vasculitis when vital organ function is already compromised, adjunctive therapies have been considered that include, plasma exchange, i.v. methylprednisolone, intravenous immunoglobulin (IVIg) and TNF blockade. […] Following the successful use of rituximab in rheumatoid arthritis and increasing appreciation of a role for B cells in the pathogenesis of AAV, several studies have reported responses to rituximab for patients with relapsing or refractory disease. […] Rituximab is now the preferred agent for refractory disease. […] A study involving 64 patients with GPA, comparing methotrexate (20 mg weekly) with leflunomide (30 mg daily) as a maintenance therapy, was terminated early due to a higher major relapse rate in the methotrexate group (46%) compared with the leflunomide group (23%). […] With the demonstration of the efficacy of rituximab in AAV, other B-cell-targeted therapies hold potential. […] There is an ongoing need for improved therapies for patients who are intolerant of current immunosuppression, and for those who pursue a relapsing or refractory course.

• #2 How We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy

  https://www.mdpi.com/2077-0383/14/1/208

  Recent studies, both retrospective and prospective, have further explored the efficacy of repeated RTX doses for maintenance therapy. […] The ideal frequency and dosage of RTX infusions, as well as the optimal duration for maintenance therapy, remain unresolved. […] Our approach to maintenance therapy once remission is achieved is schematized in Figure 1. […] Our findings suggest that for MPO-associated ANCA vasculitis, ongoing maintenance therapy may not be necessary after an effective B-cell depletion induction. […] The experience at G. Bosco Hospital supports the notion that an intensive protocol for B-cell depletion during remission induction, without routine subsequent immunosuppression, could offer a more favorable approach compared to fixed dosing of RTX, particularly in light of the delayed relapse observed in many cases.

• #2 Overview of Vasculitis – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/overview-of-vasculitis

  Less severe presentations of vasculitis may be managed with lower doses of corticosteroids, along with less potent immunosuppressants. […] Corticosteroids are tapered off or to the lowest dose that can maintain remission. For several forms of vasculitis, a less toxic immunosuppressant is prescribed to maintain remission as the corticosteroids are decreased and ideally eliminated. […] Long-term use of corticosteroids can have significant adverse effects. Patients who are taking 7.5 mg of prednisone daily or equivalent doses of other corticosteroids should be given calcium and vitamin D supplements and bisphosphonates to help prevent or minimize osteoporosis; bone density monitoring should be considered.

• #2 Vasculitis | Condition | UT Southwestern Medical Center

  https://utswmed.org/conditions-treatments/vasculitis/

  The Rheumatology Program at UT Southwestern Medical Center is recognized as among the best in the country for treating conditions such as vasculitis. Our rheumatologists provide expert, compassionate care with accurate diagnostic services and effective therapies that make a difference in patients lives. […] The goals of treatment are to reduce inflammation in the blood vessels to prevent complications and to treat the underlying disease that caused the vasculitis. At UT Southwestern, our rheumatologists develop treatment plans tailored to each patients needs. Treatment options for vasculitis begin with medications such as: […] Corticosteroids taken as pills or intravenously to relieve inflammation […] Immunosuppressants to reduce immune system activity and inflammation […] Supplements of calcium and vitamin D to help prevent bone loss that can result from long-term corticosteroid treatment. […] In rare cases, part of a wall of a blood vessel can become thin and weak, causing it to bulge outward. This condition is known as an aneurysm, which often needs surgery to repair. Patients might also need surgery to open blocked arteries.

• #2 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  The latest EULAR treatment recommendations for maintenance treatment favor rituximab over azathioprine due to its superior success record in relapse prevention. […] The current EULAR guidelines highlight the reduction of glucocorticoid exposure to improve patient wellbeing as a key goal in the management of AAV, and several of the latest recommendations are focused on achieving this. […] Evaluating success and optimizing outcomes in the management of AAV thus requires routine monitoring using a combination of assessments to evaluate the extent of AAV damage, susceptibility to comorbidities, and the impact of AAV symptoms and adverse events on the patient as well as AAV disease activity, and treatment strategy modified accordingly.

• #2 Dr. Patrick Nachman & Treating ANCA Vasculitis with Rituximab | Department of Medicine

  https://www.med.unc.edu/medicine/news/chairs-corner/podcast/treating-with-rituximab/

  In general, our approach has been to offer rituximab in patients with relatively mild to moderate severity of disease. […] The other form of treatment that is considered in those cases is called plasmapheresis where the patients blood is run through a filter to remove the offending autoantibodies that drive the inflammation. […] But for a large number of patients, rituximab is excellent induction therapy. […] The immense majority of patients really tolerate rituximab infusions very well. […] The traditional dose of rituximab was inherited from the oncology cancer treatment realm, where the dose classically used was 375 mg per meter squared body surface, weekly for four consecutive weeks. […] The big question right now, is what is the frequency of dosing of rituximab in the more long-term or maintenance phase of treatment of the disease.

• #2 Frontiers | Treatment goals in ANCA-associated vasculitis: defining success in a new era

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1409129/full

  With these shifts in treatment capabilities and understanding of patient burden, it is necessary to adjust the treatment paradigm accordingly. Treatment success is no longer defined solely by the control of disease activity; treatment success requires holistic improvement determined through the assessment of all aspects of the disease, ranging from disease control to comorbidity risk through to the assessment of health-related quality of life. […] The management of AAV has entered a new era with a strong focus on both the management and prevention of comorbidities as well as patient-reported outcomes, both of which are now considered key factors in defining treatment success. […] Immunosuppressive and anti-inflammatory treatments, mainly cyclophosphamide and prednisolone, have helped control the progression of AAV and increased the mean 5-year survival rate for patients with AAV.

• #2 Other Treatments – Vasculitis Foundation

  https://vasculitisfoundation.org/treatments-research/treatments/other-treatments/

  Treatment recommendations for vasculitis are based on the results of research studies and knowledge gained through clinical experience. However, they are just one tool to use as you and your healthcare provider build your treatment plan. Developing a treatment plan that is right for you requires careful discussion between you and your healthcare provider, guidance from research studies and treatment guidelines, and the clinical knowledge, experience and expertise of the members of your treatment team. […] While different forms of vasculitis may share similar symptoms and treatment recommendations, each disease is distinct and each person with vasculitis is unique. It is important to remember that what works for one individual may not work for you. The Vasculitis Foundation provides information on vasculitis treatments as a resource to help promote shared decision making between you and your medical team.

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