Entwicklung von Morbidität und Mortalität bei ANCA-assoziierten Vaskulitiden

Leitthema

Zusammenfassung

Das Outcome von ANCA-assoziierten Vaskulitiden (AAV) hat sich durch den Einsatz der kombinierten Immunsuppression mit Cyclophosphamid und Glukokortikoiden deutlich verbessert. Langzeitstudien haben eine Annäherung der Lebenserwartung von einzelnen „subsets“ an die Normalbevölkerung gezeigt. Innerhalb des ersten Jahres ist die Mortalität am höchsten, in erster Linie verursacht durch Infektionen. Besonders gefährdet hierdurch sind ältere Patienten mit deutlich eingeschränkter Nierenfunktion bei Diagnosestellung. Eine konsequente Pneumocystis-jiroveci-Prophylaxe und eine rasche, der Krankheitsaktivität angepasste GC-Reduktion können die Infektionsrate deutlich reduzieren. In der Langzeitbetreuung von AAV-Patienten ist auf CYC-assoziierte Spätkomplikationen (Zystitis und Malignomentwicklung) zu achten, die durch eine Uroprotektion mit Mesna und die Vermeidung hoher kumulativer CYC-Dosen auf ein Minimum reduziert werden können.

Schlüsselwörter

ANCA-assoziierte Vaskulitiden (AAV) Granulomatose mit Polyangiitis Wegener-Granulomatose Outcome Cyclophosphamid 

Development of morbidity and mortality in ANCA-associated vasculitis

Abstract

The outcome of ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis (AAV) has been significantly improved due to the combined use of cyclophosphamide (CYC) and glucocorticosteroids. Recent studies demonstrated a normalization of life expectancy for several subgroups of AAV patients. Mortality is highest in the first year after diagnosis and infections are the most frequent cause of death. Older age and renal failure are associated with worse outcome. The use of Pneumocystis jiroveci prophylaxis and subsequent activity-adapted GC dose reduction (target: below 10 mg per day) can substantially reduce the risk of severe infections. Late sequelae of CYC medication, such as cystitis and malignancy should be recognized and can be minimized by the usage of uroprotection with mesna and avoidance of high cumulative CYC doses.

Keywords

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) Granulomatosis with polyangiitis Wegener granulomatosis Outcome Cyclophosphamide 

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  1. 1.Klinik für Rheumatologie und klinische Immunologie Klinikum Bad Bramstedt und Poliklinik für RheumatologieUniversitätsklinikum Schleswig-Holstein, Campus LübeckLübeckDeutschland
  2. 2.Internistisch-rheumatologische GemeinschaftspraxisHamburgDeutschland

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