Diagnosis and management of ANCA-associated vasculitis

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

“…The treatment of AAV typically involves a combination of immunosuppressive medications to control inflammation, prevent organ damage, and induce remission. The specific treatment regimen may vary depending on the severity of the disease, organs affected, and individual patient factors [132].…”

“…The generalized and severe form of the disease (alveolar hemorrhage, glomerulonephritis, heart and CNS involvement, mononeuritis, hearing loss, and leukocytoclastic vasculitis of the skin) requires immediate initiation of induction treatment with high doses of corticosteroids and cyclophosphamide [132]. In large randomized trials, the administration of rituximab, a monoclonal antibody targeting B cells, appeared to have efficacy similar to that of cyclophosphamide [133,134].…”

“…In large randomized trials, the administration of rituximab, a monoclonal antibody targeting B cells, appeared to have efficacy similar to that of cyclophosphamide [133,134]. Cyclophosphamide may be replaced with rituximab, especially in cases where cyclophosphamide is not suitable due to high risk of toxicity or in relapsing disease [132]. Plasma exchange may be used in severe cases, particularly in patients with rapidly progressive glomerulonephritis or severe renal involvement [16,132].…”

“…Cyclophosphamide may be replaced with rituximab, especially in cases where cyclophosphamide is not suitable due to high risk of toxicity or in relapsing disease [132]. Plasma exchange may be used in severe cases, particularly in patients with rapidly progressive glomerulonephritis or severe renal involvement [16,132]. It helps remove circulating antibodies and inflammatory mediators from the bloodstream.…”

“…Once remission is achieved, a maintenance therapy regimen is typically initiated to prevent relapse and reduce corticosteroid dosage. This often involves tapering corticosteroids and using less-toxic immunosuppressive agents such as azathioprine, methotrexate, mycophenolate mofetil (MMF), or rituximab [132]. Rituximab has shown superiority over azathioprine in maintaining remission for GPA and MPA following induction therapy with either cyclophosphamide or rituximab when used in conjunction with low-dose glucocorticosteroids [139].…”