Granulomatose mit Polyangiitis und mikroskopische Polyangiitis

Zusammenfassung

Die Granulomatose mit Polyangiitis (GPA) und mikroskopische Polyangiitis (MPA) sind zwei zur ANCA-assoziierten Vaskulitis (AAV) zählende Entitäten. Beiden Erkrankungen liegt eine systemische nekrotisierende Kleingefäßvaskulitis zugrunde, die jedes Organ betreffen kann. Bei der GPA ist zusätzlich eine in der Regel den Respirationstrakt betreffende extravaskuläre nekrotisierende granulomatöse Entzündung vorzufinden. Das klinische Bild wird in der Mehrzahl der Fälle durch ein pulmorenales Syndrom mit alveolärer Hämorrhagie und rapid-progressiver Glomerulonephritis sowie weitere Organmanifestationen bestimmt. Bei der GPA imponiert zudem die fast regelhafte Mitbeteiligung des oberen Respirationstrakts. Die GPA ist mit Anti-Neutrophilen zytoplasmatischen Autoantikörpern (ANCA) mit einer Spezifität für die Proteinase 3 (PR3-ANCA) und die MPA für Myeloperoxidase (MPO-ANCA) assoziiert. Die immunsuppressive Therapie richtet sich nach der Krankheitsaktivität und Schwere der Organbeteiligung.

Abstract

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are two entities of ANCA-associated vasculitis (AAV). Both diseases are characterised by systemic necrotising small-vessel vasculitis, which can affect any organ. In GPA, extravascular necrotising granulomatous inflammation, usually affecting the respiratory tract, is found in addition. In the majority of cases, the clinical presentation is dominated by a pulmonary-renal syndrome with alveolar haemorrhage and rapidly progressive glomerulonephritis. Other organ involvement is found as well. In GPA, the upper respiratory tract is commonly affected. GPA is associated with anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for proteinase 3 (PR3-ANCA) and MPA with specificity for myeloperoxidase (MPO-ANCA). Immunosuppressive therapy depends on disease activity and the severity of organ involvement.

Schlüsselwörter

ANCA - Vaskulitis - AAV - Granulomatose mit Polyangiitis - Mikroskopische Polyangiitis

Keywords

ANCA - AAV - vasculitis - granulomatosis with polyangiitis - microscopic polyangiitis

Publication History

Received: 02 September 2022

Accepted after revision: 11 October 2022

Article published online:
11 January 2024

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