Update Ätiopathogenese der Kleingefäßvaskulitis

Translated title of the contribution: Update on etiopathogenesis of small vessel vasculitis

Sabrina Arnold, Konstanze Holl-Ulrich, Antje Müller, Sebastian Klapa, Peter Lamprecht*

*Corresponding author for this work
3 Citations (Scopus)


Small vessel vasculitis is characterized by a necrotizing inflammation of the vessel wall predominantly with involvement of small intraparenchymal arteries, arterioles, capillaries and venules. Medium-sized and occasionally large vessels can also be involved. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) are differentiated from immune complex vasculitides based on immunopathological and serological aspects. Immune complex vasculitides include IgA vasculitis, cryoglobulinemic vasculitis, hypocomplementemic urticarial vasculitis (anti-C1q vasculitis) and anti-glomerular basement membrane disease. Epidemiological and next-generation sequencing-based studies have significantly contributed to the identification of predisposing environmental factors and genetic risk factors in recent years. Under specific conditions ANCA and immune complexes can induce premature intravascular activation of neutrophilic granulocytes with degranulation and release of enzymes and reactive oxygen species, which leads to vascular damage. In granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis various factors, such as barrier dysfunction and dysbiosis of the microbiome contribute to extravascular granuloma formation predominantly affecting the respiratory tract.

Translated title of the contributionUpdate on etiopathogenesis of small vessel vasculitis
Original languageGerman
JournalZeitschrift fur Rheumatologie
Issue number4
Pages (from-to)270-279
Number of pages10
Publication statusPublished - 05.2022

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

DFG Research Classification Scheme

  • 205-18 Rheumatology

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