Department of Rheumatology and Immunology, Inselspital, University Hospital, University of Bern, Freiburgstrasse, CH-3010, Bern, Switzerland.
Department of Infectious Diseases and Hospital Hygiene, University Hospital, University of Zurich, Zurich, Switzerland.
Curr Rheumatol Rep. 2022 Oct;24(10):293-309. doi: 10.1007/s11926-022-01083-5. Epub 2022 Aug 3.
To provide a comprehensive review of drugs and neoplastic, infectious, autoinflammatory, and immunodeficiency diseases causing medium- to large-vessel vasculitis in adults with emphasis on information essential for the initial diagnostic process.
Entities with medium- to large-vessel vasculitis as clinical manifestations have been described recently (e.g., adenosine deaminase-2 deficiency, VEXAS-Syndrome), and vasculitis in established autoinflammatory or immunodeficiency diseases is increasingly being identified. In the diagnostic process of medium- to large-vessel vasculitis in adults, a large variety of rare diseases should be included in the differential diagnosis, especially if diagnosis is made without histologic confirmation and in younger patients. Although these disorders should be considered, they will undoubtedly remain rare in daily practice.
提供成人中由药物和肿瘤、感染、自身炎症和免疫缺陷疾病引起的大、中血管血管炎的全面综述，重点介绍对初始诊断过程至关重要的信息。
最近描述了具有大、中血管血管炎临床表现的实体（例如，腺苷脱氨酶 2 缺乏症、VEXAS 综合征），并且在既定的自身炎症或免疫缺陷疾病中越来越多地发现血管炎。在成人中到大血管血管炎的诊断过程中，应将多种罕见疾病纳入鉴别诊断，特别是在没有组织学证实和年轻患者的情况下进行诊断时。虽然应考虑这些疾病，但在日常实践中它们无疑仍属罕见。
