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复发性多软骨炎

Relapsing polychondritis.

作者信息

Emmungil Hakan, Aydın Sibel Zehra

机构信息

Department of Rheumatology, Koç University Faculty of Medicine, İstanbul, Turkey.

出版信息

Eur J Rheumatol. 2015 Dec;2(4):155-159. doi: 10.5152/eurjrheum.2015.0036. Epub 2015 Dec 1.

Abstract

Relapsing polychondritis (RPC) is a unique and rarely observed autoimmune condition regarded as recurrent extensive chondritis of the auricular, nasal, and tracheal cartilages. Moreover, heart, main arteries, skin, and eyes may be involved. Several forms of clinical manifestations may be seen, and the pathogenesis still remains anonymous. A concomitant disease, particularly myelodysplasia or other systemic autoimmune disease can be detected in one-third of the patients with RPC. The treatment of RPC should be considered on personal basis and classified according to disease activity and severity. This study reviews the available data on clinical manifestations, pathogenesis, diagnosis, and therapeutics of the RPC.

摘要

复发性多软骨炎(RPC)是一种独特且罕见的自身免疫性疾病,被认为是耳廓、鼻和气管软骨反复发生的广泛性软骨炎。此外,心脏、主动脉、皮肤和眼睛也可能受累。可出现多种临床表现形式,其发病机制仍不明。三分之一的RPC患者可检测到合并症,尤其是骨髓发育异常或其他全身性自身免疫性疾病。RPC的治疗应因人而异,并根据疾病活动度和严重程度进行分类。本研究回顾了有关RPC的临床表现、发病机制、诊断和治疗的现有数据。

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