Division of Rheumatology, Ibaraki Prefectural Central Hospital, Japan.
Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan.
Intern Med. 2020 Aug 1;59(15):1899-1904. doi: 10.2169/internalmedicine.4067-19. Epub 2020 Apr 23.
Cogan's syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Ménière-like attacks, is classified into "typical" and "atypical" forms, while Takayasu arteritis (TAK) is a rare large-vessel vasculitis associated with human leukocyte antigen (HLA)-B52. Very few cases meet both the CS and TAK classification criteria. We herein report a 53-year-old woman diagnosed with atypical CS and aortitis similar to TAK. Her 25-year-old daughter manifested TAK without symptoms of CS, and both are HLA-B52 positive. Our case highlights the difficulties of distinguishing aortitis with atypical CS from aortitis with TAK.
科根综合征（CS）是一种罕见的血管炎，其特征是非梅毒性间质性角膜炎和梅尼埃样发作，分为“典型”和“非典型”两种形式，而 Takayasu 动脉炎（TAK）是一种罕见的与人类白细胞抗原（HLA）-B52 相关的大血管血管炎。非常少有的病例同时符合 CS 和 TAK 的分类标准。本文报告了一例 53 岁女性，被诊断为非典型 CS 和类似于 TAK 的主动脉炎。她 25 岁的女儿表现为 TAK，无 CS 症状，且两者均为 HLA-B52 阳性。本病例强调了区分具有非典型 CS 的主动脉炎与具有 TAK 的主动脉炎的困难。
