Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.
Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
Curr Rheumatol Rep. 2021 Jul 1;23(7):49. doi: 10.1007/s11926-021-01012-y.
To provide an updated review on epidemiology, clinical manifestations, diagnostic assessment, treatment, and prognosis of localized vasculitis, following the 2012 Revised International Chapel Hill Consensus Conference Nomenclature on single-organ vasculitis.
Localized, single-organ vasculitides encompass a group of rare conditions in which there is no evidence of concomitant systemic vasculitis. Most data on this topic derives from case reports and small case series. Although some aspects of these diseases, such as clinical manifestations and histologic findings, have already been extensively investigated, there is still a lack of robust data concerning the pathogenesis, epidemiology, and treatment. Localized vasculitides may have a wide range of clinical features depending on the organ affected. The inflammatory process may have a multifocal/diffuse or unifocal distribution. Diagnosis is usually based on histopathology findings and exclusion of systemic vasculitis, which may frequently pose a challenge. Further research on treatment is warranted.
根据 2012 年修订的国际丘奇希尔共识会议单器官血管炎命名法，提供局部血管炎的流行病学、临床表现、诊断评估、治疗和预后的最新综述。
局限性、单一器官血管炎包括一组罕见疾病，其中没有同时存在系统性血管炎的证据。关于这个主题的大多数数据都来自病例报告和小病例系列。尽管这些疾病的某些方面，如临床表现和组织学发现，已经得到了广泛的研究，但关于发病机制、流行病学和治疗，仍然缺乏有力的数据。局限性血管炎可能有广泛的临床表现，取决于受影响的器官。炎症过程可能具有多灶性/弥漫性或单灶性分布。诊断通常基于组织病理学发现和排除系统性血管炎，这可能经常构成挑战。有必要进一步研究治疗方法。
