Ocular Involvement in Relapsing Polychondritis.
Ocular Involvement in Relapsing Polychondritis.
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DOI：
10.3390/jcm10214970
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发表时间：
2021-10-26
影响因子：
3.9
通讯作者：
Taniguchi Y
中科院分区：
文献类型：
--
作者：
Fukuda K;Mizobuchi T;Nakajima I;Kishimoto T;Miura Y;Taniguchi Y
复发性多软骨炎（RPC）是一种罕见的全身性免疫介导疾病，其特征是全身软骨和富含蛋白聚糖的组织反复发生炎症。耳廓、鼻腔、气管、关节软骨炎和关节炎是 RPC 患者常见的全身症状。眼部组织也是 RPC 炎症的目标，大约一半的 RPC 患者观察到多种眼部症状。巩膜炎/表层巩膜炎、葡萄膜炎和结膜炎是 RPC 相关的常见症状。较少见的是角膜炎、视网膜病变、视神经病变、肌肉麻痹和眼眶炎症。眼部炎症也可能是 RPC 的首发表现。尽管 RPC 是一种潜在致命且威胁视力的疾病，但该疾病的罕见性及其多变的临床表现可能会导致延迟诊断或误诊。鉴于 RPC 眼部受累的患病率很高，为避免误诊，医生在看到患有复发性眼部炎症和各种全身症状的患者时应怀疑 RPC。在本文中，我们对 RPC 相关的眼部表现进行了全面回顾。
Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of inflammation in RPC, and a variety of ocular symptoms are observed in approximately half of the patients with RPC. Scleritis/episcleritis, uveitis, and conjunctivitis are common symptoms associated with RPC. Less frequently, keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are also observed. Ocular inflammation could also be the first manifestation of RPC. Although RPC is a potentially fatal and sight-threatening disease, the rarity of the disease and its protean clinical presentation may lead to delayed diagnosis or misdiagnosis. Given the high prevalence of ocular involvement in RPC, to avoid misdiagnosis, physicians should be suspicious of RPC when they see patients with recurrent ocular inflammatory conditions and various systemic symptoms. In this article, we provide a comprehensive review of ocular manifestations associated with RPC.
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