Aktuelle Behandlungskonzepte ANCA-assoziierter Vaskulitiden

Zusammenfassung

Die ANCA-assoziierten Vaskulitiden (AASV) zählen zu den primär systemischen Vaskulitiden mit vorwiegendem Befall kleiner und mittelgroßer Gefäße. Pathohistologisch findet sich bei allen eine nekrotisierende pauci-immune Vaskulitis, während in der Zirkulation bei der Mehrzahl der Patienten Autoantikörper gegen zytoplasmatische Bestandteile von Neutrophilen (ANCA) nachweisbar sind. Klinisch handelt es sich bei den AASV typischerweise um mehrphasisch verlaufende entzündliche Systemerkrankungen mit häufig begleitendem rheumatischen Beschwerdekomplex. Grundsätzlich kann so gut wie jedes Organ befallen werden, wobei eine besondere Prädilektion für die Niere und die oberen und unteren Luftwege besteht. Eine Hautbeteiligung findet sich in ca. 50 % der Fälle. Moderne stadien- und schweregradadaptierte immunsuppressive Therapieprotokolle erlauben eine ca. 90 % Remissionsrate, wobei wegen der chronisch-rezidivierenden Natur der AASV grundsätzlich eine anschließende Erhaltungstherapie notwendig ist. Die komplexe Behandlung der AASV sollte in hierfür spezialisierten Zentren erfolgen.

Abstract

ANCA-associated vasculitides (AASV) belong to the group of the primary systemic vasculitides and predominantly affect small und medium-sized vessels. Pathohistologically, they are characterized by a necrotizing pauci-immune angiitis. Circulating disease-specific anti-neutrophil cytoplasmic antibodies (ANCA) can be found in the majority of these patients. AASV are clinically heterogeneous diseases which, after a common unspecific prodromal phase, lead to an overt vasculitic disease. By nature, virtually each organ may be affected, though a certain preponderance for kidney and upper and lower respiratory tract involvement is observed. Skin involvement is also found in about 50 % of affected patients. Untreated, AASV have a dismal prognosis, but current stage and severity adapted immunosuppressive regimens lead to a 90 % remission rate. Due to the chronic relapsing nature of AASV, maintenance therapy is also necessary. Due to complex treatment schemes as well as the unpredictable course of these diseases patients with AASV should be treated and followed in experienced vasculitis centers.

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