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Granulomatöse Vaskulitiden und Vaskulitiden mit extravaskulärer Granulomatose

Granulomatous vasculitides and vasculitides with extravascular granulomatosis

Zusammenfassung

Bei den Vaskulitiden handelt es sich um entzündliche Erkrankungen von Blutgefäßen, hervorgerufen durch autoimmune oder infektiöse Prozesse, die mit einer Alteration und Zerstörung der Gefäßwand einhergehen. Unter histopathologischen Gesichtspunkten werden hinsichtlich des Entzündungsmusters granulomatöse von nekrotisierenden Vaskulitiden unterschieden. Granulomatöse Vaskulitiden zeichnen sich durch intramurale vorwiegend lymphohistiozytäre Infiltrate mit Ausbildung von Riesenzellen aus. Hierzu zählen die Riesenzellarteriitis (RZA) sowie Takayasu-Arteriitis (TAK). Demgegenüber zählt die Anti-Neutrophile zytoplasmatische Autoantikörper(ANCA)-assoziierte Vaskulitis (AAV) zur Gruppe der nekrotisierenden Vaskulitiden. Die AAV umfasst die Granulomatose mit Polyangiitis (GPA), mikroskopische Polyangiitis (MPA) und eosinophile Granulomatose mit Polyangiitis (EGPA). Die GPA und EGPA sind neben der systemischen nekrotisierenden Kleingefäßvaskulitis durch eine extravaskuläre granulomatöse nekrotisierende Entzündung charakterisiert, die hauptsächlich den oberen und/oder unteren Respirationstrakt betrifft, bei EGPA überdies mit eosinophiler Komponente. Die granulomatösen Läsionen sind Teil des autoimmunen Prozesses und gehen mit Gewebsschäden einher.

Abstract

Vasculitides are inflammatory diseases of blood vessels caused by autoimmune or infectious processes, which are associated with alterations and destruction of the vascular wall. From a histopathological point of view, granulomatous vasculitides can be distinguished from necrotizing vasculitides with respect to the pattern of inflammation. Granulomatous vasculitides are characterized by intramural, predominantly lymphohistiocytic infiltrates with the formation of giant cells. They include giant cell arteritis (GCA) and Takayasu arteritis (TAK). By contrast, anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) belongs to the group of necrotizing vasculitides. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In addition to systemic necrotizing small vessel vasculitis, GPA and EGPA are characterized by extravascular granulomatous necrotizing inflammation mainly affecting the upper and/or lower respiratory tract, in EGPA with eosinophilic infiltrates. These granulomatous lesions are part of the autoimmune process and associated with tissue damage.

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Correspondence to Peter Lamprecht.

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S. Arnold, S. Klapa, K. Holl-Ulrich, A. Müller, A. Kerstein-Stähle und P. Lamprecht geben an, dass kein Interessenkonflikt besteht.

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Ina Kötter, Hamburg

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Arnold, S., Klapa, S., Holl-Ulrich, K. et al. Granulomatöse Vaskulitiden und Vaskulitiden mit extravaskulärer Granulomatose. Z Rheumatol 81, 558–566 (2022). https://doi.org/10.1007/s00393-022-01249-7

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Schlüsselwörter

  • Granulomatose mit Polyangiitis
  • Eosinophile Granulomatose mit Polyangiitis
  • Riesenzellarteriitis
  • Takayasu-Arteriitis
  • Granulom

Keywords

  • Granulomatosis with polyangiitis
  • Eosinophilic granulomatosis with polyangiitis
  • Giant cell arteritis
  • Takayasu arteritis
  • Granuloma