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进行性假类风湿性发育不良与复发性多软骨炎的罕见共存。

An Unusual Coexistence of Progressive Pseudorheumatoid Dysplasia and Relapsing Polychondritis.

作者信息

Kiliç Gamze, Kiliç Erkan, Akgül Özgür, Atakul Nimet, Özgöçmen Salih

机构信息

Department of Physical Medicine and Rehabilitation, Division of Rheumatology, Medical Faculty of Erciyes University, Kayseri, Turkey.

出版信息

Arch Rheumatol. 2016 Apr 13;31(3):290-294. doi: 10.5606/ArchRheumatol.2016.5994. eCollection 2016 Sep.

Abstract

Progressive pseudorheumatoid dysplasia is a rare hereditary skeletal disease characterized by bone and cartilage dysplasia, progressive arthropathy without sign of systemic or synovial inflammation. Relapsing polychondritis (RP) is a rare autoimmune disease associated with inflammation in cartilaginous and other proteoglycan rich structures. An associated autoimmune and/or hematologic disorder is seen in over 30% of patients with RP. To our knowledge, coexistence of progressive pseudorheumatoid dysplasia and RP has not been previously reported. In this article, we describe an unusual case of coexisting progressive pseudorheumatoid dysplasia with RP. Physicians should be aware of this possible association.

摘要

进行性假类风湿性发育不良是一种罕见的遗传性骨骼疾病,其特征为骨和软骨发育异常、进行性关节病且无全身或滑膜炎症迹象。复发性多软骨炎(RP)是一种罕见的自身免疫性疾病,与软骨及其他富含蛋白聚糖的结构炎症相关。超过30%的RP患者伴有自身免疫和/或血液系统疾病。据我们所知,进行性假类风湿性发育不良与RP并存此前尚未见报道。在本文中,我们描述了一例进行性假类风湿性发育不良与RP并存的罕见病例。医生应意识到这种可能的关联。

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