musculoskeletal and connective tissue disorders
Overview of Vasculitis
Treatment of Vasculitis
Induction of remission for life-threatening or organ-threatening vasculitis with corticosteroids, often withcyclophosphamideorrituximab Induction of remission for less severe vasculitis with corticosteroids plus a less potent immunosuppressant (eg,methotrexate,azathioprine,mycophenolatemofetil) orrituximab Maintenance of remission withmethotrexate,azathioprine, orrituximab, plus tapering of corticosteroids Treatment of vasculitis depends on the etiology, the type of vasculitis, and extent and severity of disease. For secondary vasculitic disorders, removing the cause (eg, infection, drug, cancer) usually helps. For primary vasculitic disorders, treatment aims to induce and maintain remission. Remission is induced by using cytotoxic immunosuppressants and high-dose corticosteroids, usually for 3 to 6 months, until remission occurs or disease activity is acceptably reduced. The duration of remission is hard to predict and may depend on the type of vasculitis. For many patients, maintaining remission requires continuation of immunosuppressive therapy with or without a low dose of corticosteroids. During this period, the goal is to eliminate corticosteroids or reduce their dose and use alternative, less toxic immunosuppressants as long as needed. All patients treated with immunosuppressants should be monitored for opportunistic and other infections. Testing for tuberculosis and hepatitis B, which can become reactivated by some immunosuppressive therapies, should be done. Prevention should be considered for patients receiving potent or prolonged immunosuppressive therapy. For less severe forms of vasculitis, low doses of corticosteroids and less potent immunosuppressants (eg,methotrexate,azathioprine,mycophenolatemofetil) may be used. 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. Treatment typically consists of the following: Corticosteroids:High-dose corticosteroids (also called pulse corticosteroids) are often prescribed. Specific doses and drugs must be individualized. As one example,methylprednisolone15 mg/kg or 1 g IV once a day for 3 days may be used, followed by 1 mg/kgprednisoneormethylprednisoloneorally (or, if hospitalized, sometimes IV) once a day for about 4 weeks. The dose is then tapered slowly, as tolerated, until the drug is stopped. Changes in tapering schedule may be necessary if the patient fails to improve or relapses. , should also be given Prevention . Mesna:Mesnais mixed with IVcyclophosphamideto bind acrolein, a product ofcyclophosphamidedegradation that is toxic to the bladder epithelium and can lead to hemorrhagic cystitis and sometimes transitional cell carcinoma of the bladder. Long-term use ofcyclophosphamideincreases the risk of bladder cancer. One milligram ofmesnais added for each milligram ofcyclophosphamide. Recurrence of hematuria, especially without casts and dysmorphic red cells, should prompt a referral for urologic evaluation. Cystoscopy and renal imaging should be done to exclude cancer. IV once a week for 4 weeks. A widely used alternative regimen is two 1000-mg infusions given 2 weeks apart. Patients should also be given Prevention . B cell-depleting therapies will markedly blunt the response to vaccines for months after administration. Corticosteroids are tapered to zero or to the lowest dose that can maintain remission. For some forms of vasculitis (most clearly demonstrated in ANCA-associated disease), weeklymethotrexate(with folate) or dailyazathioprineis prescribed to replacecyclophosphamidebecause these drugs have a better adverse effects profile. Periodic IVrituximabmay also be used to maintain remission, but the optimal dosage and infusion interval have not been clearly established. The duration of this treatment varies, from one year to several years, depending on the patient, specific diagnosis, and propensity for relapse. Patients with frequent relapses may need to take immunosuppressants indefinitely. supplements and bisphosphonates to help Treatment ; , a selective C5a receptor antagonist, is an available adjunctive therapy for severe active ANCA-associated vasculitis ( Treatment reference ). 1.Jayne DRW, Merkel PA, Schall TJ, et al:Avacopanfor the treatment of ANCA-associated vasculitis.N Engl J Med384(7):599-609, 2021. doi: 10.1056/NEJMoa2023386. PMID: 33596356 