Nierenbeteiligung bei systemischen Vaskulitiden

Zusammenfassung

Systemische Vaskulitiden sind eine heterogene Gruppe von Autoimmunerkrankungen, in deren Verlauf durch entzündliche Gefäßveränderungen eine Gewebszerstörung und ein Funktionsverlust der betroffenen Organe auftreten. Die Einteilung der Vaskulitiden erfolgt nach ätiologischen, morphologischen und immunologischen Kriterien. Neben der Unterscheidung zwischen primären und sekundären Vaskulitisformen, die im Rahmen anderer Erkrankungen auftreten, unterteilt man nach den betroffenen Gefäßabschnitten in Klein-, Mittel- und Großgefäßvaskulitiden. Pathogenetisch bedeutsam ist die Abgrenzung der Immunkomplex bedingten gegenüber den pauci-immunen Vaskulitiden. Tritt im Rahmen einer systemischen Vaskulitis eine Beteiligung der Nieren auf, so ist die rapid progressive Glomerulonephritis (RPGN) mit einem Verlust der Organfunktion eine bedrohliche Komplikation und bedarf einer raschen Diagnostik und Therapie. Das charakteristische histologische Bild einer ANCA-assoziierten Vaskulitis ist eine schwere Entzündungsreaktion in Form halbmondbildender glomerulärer Nekrosen und eine Vaskulitis der kleinen Gefäße. Es liegen evidenzbasierte Therapieprotokolle für die Remissionsinduktion und Remissionserhaltung vor. Grundsätzlich wird initial eine höherpotente Immunsuppression, bei kritischer Organbeteiligung mit Prednisolon und Cyclophosphamid durchgeführt. Bei refraktären Verläufen ist die Behandlung mit einem CD20-Antikörper (Rituximab) etabliert. Zur Remissionserhaltung kommen anschließend Azathioprin, Methotrexat oder Leflunomid in Betracht. Von klinischer Bedeutung ist die Vermeidung erkrankungs- und therapieassoziierter Komplikationen (Infektionen, gonadale Insuffizienz bei Cyclophosphamidtherapie, Steroid-induzierte Osteoporose und Atherosklerose). Auch immunkomplexbedingte Vaskulitiden können zu einer Nierenbeteiligung (RPGN) führen, die jedoch meist weniger aggressiv verläuft. Für die Kryoglobulinämie ist eine Assoziation zu einer aktiven Hepatitis C bekannt, wobei die erfolgreiche Therapie der Virusinfektion zu einer Remission der Vaskulitis führt. Großgefäßvaskulitiden manifestieren sich meist nur indirekt an den Nieren. Eine Beteiligung der Nierenarterien und eine konsekutive renale Hypertonie sind möglich.

Abstract

The systemic vasculitides are a heterogenous group of autoimmune disorders, characterised by vessel wall inflammation leading to tissue damage and consecutive loss of function in the dependent organ systems. Vasculitides are classified by aetiological, morphological and immunological criteria. Primary and secundary vasculitis are distinguished diseases and they are categorised according to different sizes of the involved vessels into small, medium and large vessel vasculitis. Immunologically important are 2 different types of vasculitis, immune complex-mediated and pauci immune vasculitis. Renal involvement with rapid progressive glomerulonephritis (RPGN) is a life-threatening complication and needs to be diagnosed and treated immediately. Necrotising crescentic glomerulonephritis is histologically characteristic for ANCA-associated vasculitis. There are evidence-based protocols for induction and maintenance therapy. Combined treatment with steroids and cyclophosphamide is mandatory for critical organ involvement. B-cell-depleting therapy with rituximab is an efficient alternative. Azathioprine, methotrexate and leflunomide are recommended for maintaining remission. Infections, related to therapy or disease, are of particular clinical interest, because they are responsible for increased mortality during the early course of disease. Furthermore, gonadal insufficiency after cyclophosphamide therapy and steroid-induced osteoporosis should be prevented. Immune complex-mediated vasculitis could also cause renal involvement, although the course of glomerulonephritis is less agressive. Kryoglobulinaemic vasculitis is often associated with active hepatitis C infection and remission could be achieved by elimination of virus with interferon alpha. Renal involvement in large vessel vasculitis is mainly attributed to ischaemia and consecutive renal hypertension.

Schlüsselwörter

systemische Vaskulitis - Nierenbeteiligung - Pathogenese - Therapie

Key words

systemic vasculitis - renal involvement - pathogenesis - therapy

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