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Neues zur Pathogenese primär systemischer Immunvaskulitiden

New pathogenetic aspects in primary systemic vasculitides

  • Schwerpunkt: Generalisierte und organbezogene Autoimmunerkrankungen
  • Published:
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Zusammenfassung

Primär systemische Vaskulitiden werden aufgrund immunpathologischer Merkmale und entsprechend der Größe der befallenen Gefäße eingeteilt. Den 3 Kleingefäßvaskulitiden, die mit antineutrophilen zytoplasmatischen Autoantikörper (ANCA) assoziiert sind (Wegenersche Granulomatose, Churg-Strauss-Syndrom, mikroskopische Polyangiitis), stehen die sog. Nicht-ANCA-assoziierten Vaskulitiden gegenüber. Dies sind die granulomatösen Arteriitiden großer Gefäße (Riesenzellarteriitis, Takayasu-Arteriitis) und die Immunkomplexvaskulitiden mittelgroßer und kleiner Gefäße (Polyarteriitis nodosa, Kawasaki-Erkrankung und Henoch-Schönlein-Purpura, kryoglobulinämische Vaskulitis, kutane leukozytoklastische Angiitis). Prädisponierende genetische und weitere endogene und exogene Faktoren begünstigen eine Aktivierung der angeborenen bzw. natürlichen Immunität und induzieren über persistierende Entzündungsreaktionen die unterschiedlichen Formen der (Auto-)Immunvaskulitiden.

Abstract

Primary systemic vasculitides are defined according immunopathological features and the size of the involved vessels. Three anti-neutrophil cytoplasmic autoantibody (ANCA) associated small vessel vasculitides (Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis) can be distinguished from the so-called Non-ANCA-associated vasculitides, i.e. granulomatous vasculitides of large vessels (giant cell arteritis, Takayasu arteritis) and immune complex-mediated vasculitides of medium-sized and small vessels (Polyarteriitis nodosa, Kawasaki disease and Henoch-Schönlein purpura, cryoglobulinemic vasculitis, cutaneous leukocytoklastische angiitis). Predisposing genetic and other endogenous and exogenous factors facilitate the activation of innate immunity and induce persisting inflammatory reactions resulting in different forms of (auto)-immune vasculitides.

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Danksagung

Mein Dank gilt der Deutschen Forschungsgemeinschaft für die Unterstützung durch die Klinische Forschergruppe (KFO) 170 „Frühpathogenese der Wegenerschen Granulomatose: Von der natürlichen Abwehr mit Granulombildung zur Autoimmunität“ und dem Exzellenzcluster „Inflammation at Interfaces“ Research Area I-h.

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  1. Poliklinik für Rheumatologie, Vaskulitiszentrum UKSH & Rheumaklinik Bad Bramstedt, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland

    P. Lamprecht

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Lamprecht, P. Neues zur Pathogenese primär systemischer Immunvaskulitiden. Internist 50, 291–297 (2009). https://doi.org/10.1007/s00108-008-2300-z

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