Wegener-Granulomatose und mikroskopische Polyangiitis

摘要: Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides, associated with a positive C/PR3-ANCA in WG P/MPO-ANCA MPA. The most prominently involved organs the upper (only WG) lower respiratory tract kidneys. diagnostic work-up is an interdisciplinary approach assessing disease stage extent. Treatment adapted to extent relies on combination of cytotoxic plus tapering regimen corticosteroids. Induction remission "early systemic" can be achieved low-dose methotrexate. In severe generalized vasculitis cyclophosphamide (CYC) mainstay therapy, rapidly progressive glomerulonephritis plasmapheresis. After 3-6 months induction CYC switched maintenance treatment azathioprine. Alternatives leflunomide, mycophenolate or methotrexate (creatinine = 50 at diagnosis, renal dysfunction pulmonary involvement higher mortality rates. relapse rate approximately 50% within 5 years, being than