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Wegener-Granulomatose und mikroskopische Polyangiitis

Wegener’s granulomatosis and microscopic polyangiitis

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Zusammenfassung

Die Wegener-Granulomatose (WG) und mikroskopische Polyangiitis (MPA) sind primäre systemische Kleingefäßvaskulitiden, die mit dem Nachweis eines C-ANCA/PR3-ANCA bei der WG und P-ANCA/MPO-ANCA bei der MPA assoziiert sind. Klinisch führend sind Beteiligungen des oberen (nur bei WG) und unteren Respirationstraktes und der Nieren. Die Diagnostik zur Festlegung von Krankheitsausdehnung und -aktivität ist interdisziplinär. Die Therapie erfolgt stadien- und aktivitätsadaptiert. Eine Remissionsinduktion bei der „Early-systemic-WG/MPA“ kann mit Methotrexat (MTX) erfolgen. Bei schwer generalisierten Verläufen ist Cyclophosphamid (CYC) unverzichtbar, bei rapid progressiver Glomerulonephritis (RPGN) mit zusätzlicher Plasmapheresebehandlung. Nach etwa 3- bis 6-monatiger CYC-Therapie wird auf eine remissionserhaltende Therapie mit Azathioprin umgestellt. Alternativen sind Leflunomid, Mycophenolat oder MTX (Kreatinin <150 µmol/l). Alter ≥50 Jahre, Lungenbeteiligung oder Nierenfunktionseinschränkung bei Diagnose sind mit einer erhöhten Mortalität assoziiert. Die Rezidivrate liegt um 50% in 5 Jahren, bei der WG höher als bei der MPA.

Abstract

Wegener’s granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides, associated with a positive C/PR3-ANCA in WG and P/MPO-ANCA in MPA. The most prominently involved organs are the upper (only in WG) and lower respiratory tract and the kidneys. The diagnostic work-up is an interdisciplinary approach assessing disease stage and extent. Treatment is adapted to disease stage and extent and relies on a combination of a cytotoxic plus a tapering regimen of corticosteroids. Induction of remission in “early systemic” disease can be achieved with low-dose methotrexate. In severe generalized vasculitis cyclophosphamide (CYC) is the mainstay of therapy, in rapidly progressive glomerulonephritis in combination with plasmapheresis. After 3–6 months of induction CYC is switched to a maintenance treatment with azathioprine. Alternatives are leflunomide, mycophenolate or methotrexate (creatinine < 150 µmol/l). Age ≥ 50 at diagnosis, renal dysfunction and pulmonary involvement are associated with higher mortality rates. The relapse rate is approximately 50% within 5 years, being higher in WG than MPA.

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Interessenkonflikt

Die korrespondierende Autorin weist auf folgende Beziehungen hin: Eva Reinhold-Keller: Referententätigkeit Fa. Abbott, Wyeth, Genzyme, Sanofi Aventis. Kirsten de Groot: Referententätigkeit Fa Hoffmann-La Roche, Amgen, Abbot, Wyeth, Janssen-Cilag.

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  1. Medizinische Klinik III (Innere Medizin, Nephrologie, Rheumatologie), Klinikum Offenbach GmbH, Starkenburgring 66, 63069, Offenbach, Deutschland

    K. de Groot

  2. Internistisch-rheumatologische Gemeinschaftspraxis, Hamburg/Klinikum Bad Bramstedt, Hamburg, Deutschland

    E. Reinhold-Keller

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de Groot, K., Reinhold-Keller, E. Wegener-Granulomatose und mikroskopische Polyangiitis. Z. Rheumatol. 68, 49–64 (2009). https://doi.org/10.1007/s00393-008-0425-z

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