Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides

Peter Lamprecht, Anja Kerstein, Sebastian Klapa, Susanne Schinke, Christian M Karsten, Xinhua Yu, Marc Ehlers, Jörg T Epplen, Konstanze Holl-Ulrich, Thorsten Wiech, Kathrin Kalies, Tanja Lange, Martin Laudien, Tamas Laskay, Timo Gemoll, Udo Schumacher, Sebastian Ullrich, Hauke Busch, Saleh Ibrahim, Nicole FischerKatrin Hasselbacher, Ralph Pries, Frank Petersen, Gesche Weppner, Rudolf Manz, Jens Y Humrich, Relana Nieberding, Gabriela Riemekasten, Antje Müller


Anti-neutrophil cytoplasmic autoantibodies (ANCA) targeting proteinase 3 (PR3) and myeloperoxidase expressed by innate immune cells (neutrophils and monocytes) are salient diagnostic and pathogenic features of small vessel vasculitis, comprising granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic GPA. Genetic studies suggest that ANCA-associated vasculitides (AAV) constitute separate diseases, which share common immunological and pathological features, but are otherwise heterogeneous. The successful therapeutic use of anti-CD20 antibodies emphasizes the prominent role of ANCA and possibly other autoantibodies in the pathogenesis of AAV. However, to elucidate causal effects in AAV, a better understanding of the complex interplay leading to the emergence of B lymphocytes that produce pathogenic ANCA remains a challenge. Different scenarios seem possible; e.g., the break of tolerance induced by a shift from non-pathogenic toward pathogenic autoantigen epitopes in inflamed tissue. This review gives a brief overview on current knowledge about genetic and epigenetic factors, barrier dysfunction and chronic non-resolving inflammation, necro-inflammatory auto-amplification of cellular death and inflammation, altered autoantigen presentation, alternative complement pathway activation, alterations within peripheral and inflamed tissue-residing T- and B-cell populations, ectopic lymphoid tissue neoformation, the characterization of PR3-specific T-cells, properties of ANCA, links between autoimmune disease and infection-triggered pathology, and animal models in AAV.

Original languageEnglish
JournalFrontiers in Immunology
Pages (from-to)680
Publication statusPublished - 2018

Research Areas and Centers

  • Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)


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