Vaskulitiden mit pulmonaler Beteiligung

 

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Zusammenfassung

Die vielfältigen pulmonalen Manifestationen von Vaskulitiden sind eine große diagnostische und therapeutische Herausforderung. Eine Vaskulitis sollte bei schnellen, schweren und ungewöhnlichen Verläufen von Lungenfunktionseinschränkungen in Betracht gezogen werden. Die klinische Untersuchung häufig ebenfalls betroffener Organsysteme, insbesondere Haut und Niere auf der einen und die spezifischen Antikörperuntersuchungen auf der anderen Seite, sind komplementäre Ansätze zur Diagnosesicherung.

Die Lunge ist häufig bei Kleingefäßvaskulitiden wie der anti-GBM-Vaskulitis/Goodpasture-Syndrom und den ANCA-assoziierten Vaskulitiden beteiligt. Lebensbedrohliche Blutungen und schnelle, irreversible Schäden anderer Organe, häufig der Niere, sind gefürchtete Komplikationen, sodass eine schnelle Diagnose nötig ist.

Angesichts der Seltenheit der Erkrankungen, vieler neuer Medikamente einschließlich Biologicals und häufig multipler betroffener Organe sind der Kontakt zu einem spezialisierten Zentrum und Kooperation der Fachabteilungen zentral, um das optimale Behandlungsergebnis für die betroffenen Patienten zu erreichen. Alle betreuenden Ärzte sollten die Nebenwirkungsspektren der immunsuppressiven Induktions- und Erhaltungstherapien kennen.

Abstract

The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation.

Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides. Life threatening pulmonary hemorrhage and irreversible damage of other organs, frequently the kidney, are important complications necessitating rapid diagnosis of these conditions.

Vasculitides are rare diseases of multiple organs and therapies including biologics are evolving rapidly, requiring cooperation of specialities and with specialized centres to achieve best patient care. All involved physicians should be aware of typical complications of immunosuppressive therapy.

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