Arnold Sabrina, Klapa Sebastian, Holl-Ulrich Konstanze, Müller Antje, Kerstein-Stähle Anja, Lamprecht Peter
Klinik für Rheumatologie und klinische Immunologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.
Pathologie - Hamburg, MVZ Labor Lademannbogen GmbH, Hamburg, Deutschland.
Z Rheumatol. 2022 Sep;81(7):558-566. doi: 10.1007/s00393-022-01249-7. Epub 2022 Aug 12.
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.
血管炎是由自身免疫或感染过程引起的血管炎症性疾病，与血管壁的改变和破坏有关。从组织病理学角度来看，肉芽肿性血管炎可根据炎症模式与坏死性血管炎区分开来。肉芽肿性血管炎的特征是壁内主要为淋巴细胞组织细胞浸润并形成巨细胞。它们包括巨细胞动脉炎（GCA）和大动脉炎（TAK）。相比之下，抗中性粒细胞胞浆自身抗体（ANCA）相关血管炎（AAV）属于坏死性血管炎组。AAV包括肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA）。除系统性坏死性小血管血管炎外，GPA和EGPA的特征是血管外肉芽肿性坏死性炎症，主要影响上呼吸道和/或下呼吸道，在EGPA中伴有嗜酸性粒细胞浸润。这些肉芽肿性病变是自身免疫过程的一部分，并与组织损伤有关。
