Granulumatosis with polyangiitis (wegener’s)/GPA microscopic polyangiitis (MPA) and Churg Strauss syndrome (CSS) are
primary systemic vasculitides which predominantly affect small vessels, showing a high association with a positive C/PR3-ANCA in
GPA and P/MPO-ANCA in MPA, so called ANCA-associated vasculitides (AAV). The diagnostic work-up relies on an interdisciplinary
approach including imaging techniques and laboratory tests in order to assess disease stage and extent. The golden standard remains the
histological proof of a necrotizing, pauci-immune small vessel vasculitis, in GPA additionally non-caseating granuloma is found mainly
in the respiratory tract. Treatment is adapted to disease stage and extent and relies on a combination of a cytotoxic plus a tapering regimen
of glucocorticosteroids. Induction of remission in “early systemic” disease without organ- and life-threatening organ manifestations
and a near normal kidney function can be achieved with methotrexate. In the generalized phase with significant renal dysfunction cyclophosphamide
is the mainstay of therapy, in rapidly progressive glomerulonephritis with an imminent dialysis indication plasmapheresis is
performed additionally. When remission is achieved, usually after 3-6 months of induction treatment, cyclophosphamide is switched to
azathioprine as maintenance of remission drug. Alternative therapies are methotrexate provided the kidney function is normal or Leflunomide
in the long-term follow-up the relapse rate in ANCA-associated vasculitis is approximately 50% in 5 years, irrespective of the
drug used for maintenance treatment. The relapse rate is significantly higher in GPA than in MPA and CSS.
Keywords: Granulumatosis with polyangiitis (wegener’s), antineutrophil cytoplasmic antibody, ANCA, cyclophosphamide, rituximab, Churg Strauss syndrome (CSS), granuloma, methotrexate, renal dysfunction, azathioprine.
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