Granulomatose mit Polyangiitis

Abstract

Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.

Teaser

Die Granulomatose mit Polyangiitis (GPA) ist eine seltene Erkrankung ungeklärter Ätiologie, die lebensbedrohlich verlaufen kann. Bei der immunsuppressiven Therapie sind jedoch in den letzten Jahren deutliche Fortschritte zu verzeichnen. Der Beitrag schildert die wichtigsten Erkenntnisse zu Pathogenese und Histomorphologie und fasst die aktuellen Empfehlungen zu Diagnostik und Therapie zusammen.

Schlüsselwörter

Granulomatose mit Polyangitiis - ANCA - Proteinase 3 - Nekrose - Entzündung

Key words

Granulomatosis with polyangitiis - ANCA - Proteinase 3 - Nekrosis - Inflammation

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