Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
Semin Respir Crit Care Med. 2018 Aug;39(4):425-433. doi: 10.1055/s-0038-1669412. Epub 2018 Nov 7.
Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. In the lung, vasculitis impacts both the pulmonary vessels and parenchyma. Extrapulmonary involvement, particularly with concomitant kidney involvement, is a frequent occurrence. Pulmonary vasculitides often present with hemoptysis, pathologically manifested as diffuse alveolar hemorrhage (DAH) with or without capillaritis and can be life threatening when diffuse throughout the lungs. Etiologies for DAH include both primary and secondary vasculitides, along with collagen-vascular diseases, infection, and drug toxicity. Therefore, diagnosing the specific vasculitic etiology often relies on comprehensive assessment of all clinical, laboratory/serological, imaging, and histopathologic features that may be present. The most common primary pulmonary vasculitides often affect small vessels and are associated with circulating antineutrophilic cytoplasmic antibodies (ANCAs). In the 2012 classification, these include granulomatosis with polyangiitis (formerly Wegener granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss' syndrome), and microscopic polyangiitis. Other less frequent vasculitides that are non-ANCA associated or affect medium- to large-sized vessels can have pulmonary involvement. These entities are usually associated with extrapulmonary disease and include polyarteritis nodosa, Takayasu's arteritis, Behçet's disease, and antibasement membrane antibody disease (formerly Goodpasture's syndrome). Although all vasculitides have vessel wall inflammation at some phase in the disease process, their histopathologic findings are as diverse as the group of diseases themselves. The characteristic histologic findings of the pulmonary vasculitides will be reviewed here.
血管炎是一组异质性疾病,其特征为至少在疾病过程的某个阶段存在血管壁炎症。血管炎可影响许多解剖部位的任何口径或类型的血管;然而,疾病不仅会改变血管。鉴于血管炎的多样性,2012 年提出了一种修订的分类系统,根据受累血管的类型(包括大小、功能和结构属性)对血管炎进行分类。在肺部,血管炎会影响肺部血管和实质。肺部以外的累及,特别是伴有肾脏同时受累,是常见的。肺部血管炎常表现为咯血,病理学表现为弥漫性肺泡出血(DAH),伴或不伴毛细血管炎,当弥漫性累及肺部时可危及生命。DAH 的病因包括原发性和继发性血管炎、胶原血管疾病、感染和药物毒性。因此,诊断特定血管炎的病因通常依赖于对所有可能存在的临床、实验室/血清学、影像学和组织病理学特征进行全面评估。最常见的原发性肺部血管炎常影响小血管,并与循环抗中性粒细胞胞质抗体(ANCAs)相关。在 2012 年的分类中,这些包括肉芽肿性多血管炎(以前称为韦格纳肉芽肿病)、嗜酸性肉芽肿性多血管炎(以前称为变应性肉芽肿性血管炎)和显微镜下多血管炎。其他不太常见的非 ANCAs 相关或影响中至大血管的血管炎也可能有肺部受累。这些实体通常与肺部疾病有关,包括结节性多动脉炎、大动脉炎、贝赫切特病和抗基底膜抗体病(以前称为Goodpasture 综合征)。尽管所有血管炎在疾病过程的某个阶段都有血管壁炎症,但它们的组织病理学发现与疾病本身一样多种多样。本文将回顾肺部血管炎的特征性组织病理学发现。
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