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复发性多软骨炎的临床表现

Author
Clement J Michet, MD
Section Editor
Peter H Schur, MD
Deputy Editor
Paul L Romain, MD
Translators
王炎焱, 副主任医师

引言

复发性多软骨炎(relapsing polychondritis, RPC)是一种免疫介导疾病，与软骨结构内及其他全身组织的炎症相关，尤其是耳、鼻、眼、关节和呼吸道。约1/3的RPC患者并发有另一疾病，通常是某种形式的系统性血管炎、某种结缔组织病或骨髓增生异常综合征(myelodysplastic syndrome, MDS)。

RPC的临床特征和病程在不同的患者中差异很大。细微的早期表现常在很长一段时间里不会被发现。因此，常常在出现RPC的典型特征(如，耳部炎症、鞍鼻畸形或软骨破坏的其他特征)后才做出诊断。并无已知的临床或实验室检查可预测该病的具体临床表现或总体病程。

本专题将总结RPC的临床特征。RPC的诊断、治疗、发病机制和病理学将单独讨论。(参见“复发性多软骨炎的诊断性评估”和“复发性多软骨炎的治疗”和“复发性多软骨炎的病因与发病机制”和“复发性多软骨炎的病理学”)

流行病学

关于RPC的流行病学还有很多信息未知。该病在任何年龄和任何人种中均可发病，且男女受累比例相同。白种人的RPC患病率最高。据估计，美国明尼苏达州罗切斯特市的发病率为每年3.5人/百万人[1]。RPC的好发年龄为40-60岁，但也可发生于儿童期(图 1)[2]。RPC并不是家族病，但有研究表明可能存在一些促成该病易感性的遗传因素。一项病例报告提出存在胎盘传播的可能性[3]；然而，并无其他病例证实这一现象，且所报道的这种关联性可能是偶然发生或是其他因素共同作用的结果。总的来说，即使是可能正处于多系统疾病活动期的妊娠女性，出现提示RPC经胎盘传播的情况也异常罕见[3]。

临床表现

在患者起病时和在整个病程中，RPC的临床特征在严重程度和持续时间方面都不同。耳部受累是最常见的特征，但其他解剖区域和器官也可能受累，包括：肋软骨、眼、鼻、气道、心脏、血管系统、皮肤、关节、肾脏和神经系统(表 1)。据推测，血管疾病是RPC的许多主要临床表现的原因[4-6]。还可能出现非特异性全身症状，例如乏力、不适和发热；RPC也可表现为不明原因发热。

                  

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Literature review current through: 2017-06 . | This topic last updated: 2017-01-09.
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