Nierenbeteiligung bei ANCA-assoziierten Vaskulitiden

Zusammenfassung

ANCA-assoziierte Vaskulitiden sind seltene Kleingefäßvaskulitiden, wobei die jährliche Inzidenz in etwa bei 20 pro 1 000 000 Einwohner liegt. Eine Nierenbeteiligung kommt bei über 50% der betroffenen Patienten vor und geht mit Morbidität und Mortalität einher. Ein Screening auf Nierenbeteiligung sollte zum Zeitpunkt der Erstdiagnose und periodisch im Verlauf stattfinden. Eine Nierenbiopsie hat nicht nur einen diagnostischen Nutzen, sondern ist essentiell zur Prognoseabschätzung. Verschiedene histologische Kriterien sind in den letzten Jahren entwickelt worden, welche in den klinischen Alltag Einzug finden werden. Neben glomerulären Veränderungen scheinen für die Langzeitprognose auch tubulointerstitielle Veränderungen von Interesse zu sein. Die Therapie der ANCA-assoziierten Vaskulitis, sowohl in der Induktions- als auch in Erhaltungstherapie, wurde durch Rituximab verändert. Strategien zur Glucocorticoidreduktion bzw. -elimination wurden entwickelt, welche die Nebenwirkungen von Steroiden minimieren sollen. Da eine eingeschränkte Nierenfunktion das kardiovaskuläre Risiko von Patienten mit ANCA-assoziierter Vaskulitis deutlich erhöht, muss auf eine adäquate Kontrolle dieser Faktoren geachtet werden. Zudem haben Patienten während aktiver Phasen der Erkrankung ein hohes thromboembolisches Risiko und bei dementsprechenden Beschwerden sollte frühzeitig ein Screening stattfinden. Zusammengefasst hat das zunehmende wissenschaftliche Interesse auf dem Gebiet der Vaskulitiden dazu geführt, dass die Diagnostik und Therapie über das letzte Jahrzehnt deutlich optimiert wurde. Weitere Studien werden in der Zukunft „maßgeschneiderte“ therapeutische Behandlungen erlauben.

Abstract

ANCA-associated vasculitides are rate small vessel vasculitides. The annual incidence in Europe is approximately 20 per million inhabitants. Renal involvement is observed in over 50% of patients and is linked with increased morbidity and mortality. Screening for renal involvement is recommended during initial investigation and should be performed periodically afterwards. A kidney biopsy has a diagnostic purpose, but is also helpful to predict prognosis. Different histologic criteria have been developed during the past years, which have been already incorporated in scoring kidney specimens. Despite glomerular changes tubulointerstitial changes are of interest to predict long-term renal function. Therapeutically, rituximab changed the landscape in both the induction and the maintenance phase. Strategies to minimize or eliminate steroids will find their way into clinical routine to reduce the detrimental side effects of steroids. Impaired renal function increases the cardiovascular risk of patients with ANCA-associated vasculitis, thus control of cardiovascular risk factors seems essential in this context. During phases of active disease patients are hypercoagulable and prone to thromboembolic events. Patients at risk and with symptoms should undergo prompt screening. Taken together, this review summarizes the progress in diagnostic and therapeutic approaches during the last decade. We are facing a bright future with a likely implemented “tailored and personalized” medicine in ANCA-associated vasculitis.

Schlüsselwörter

Vaskulitis - ANCA - Rituximab - Cyclophosphamid - Niere - Prognose

Key words

Vasculitis - ANCA - rituximab - cyclophosphamide - kidney - prognosis

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