Motozawa Naohiro, Nakamura Takahiro, Takagi Seiji, Fujihara Masashi, Hirami Yasuhiko, Ishida Kazuhiro, Sotozono Chie, Kurimoto Yasuo
Department of Ophthalmology, Kobe City Medical Center General Hospital Department of Ophthalmology, Institute of Biomedical Research and Innovation Hospital, Kobe Department of Frontier Medical Science and Technology for Ophthalmology Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Medicine (Baltimore). 2017 Oct;96(41):e7951. doi: 10.1097/MD.0000000000007951.
Relapsing polychondritis (RP) is a rare collagen disease characterized by inflammation and destruction of cartilage throughout the body. The paper details the clinical course of a case of RP with unique circumferential peripheral keratitis.
A 54-year-old Japanese woman was referred to the hospital presenting with auricular and ocular pain.
Based on the auricle biopsy results and the three presenting symptoms (bilateral auricular chondritis, inflammatory arthritis and ocular inflammation), her condition was diagnosed as RP.
The three presenting symptoms gradually improved with prednisolone (PSL), methylprednisolone and cyclophosphamide combination therapy, followed by PSL, methotrexate and infliximab combination therapy. However, one month after the initial visit, despite ongoing treatment, a unique circumferential peripheral keratitis suddenly occurred, in which the corneal infiltration gradually clumped together and shrank at the peripheral area. The eye and ear pain showed exacerbations and remissions on reducing the dosage of steroid drugs. The general condition was improved on altering systemic therapy to PSL, methotrexate and tocilizumab.
Keratitis gradually disappeared within 10 months of the initial visit.
This is the first report of a case of RP causing unique circumferential peripheral keratitis. This keratitis occurred despite use of focal and systemic steroids and showed improvement with general recovery. This may indicate that stabilization of general condition is important for recovery from keratitis in RP.
复发性多软骨炎（RP）是一种罕见的胶原病，其特征是全身软骨发生炎症和破坏。本文详细介绍了一例伴有独特的环形周边角膜炎的RP患者的临床病程。
一名54岁的日本女性因耳部和眼部疼痛被转诊至我院。
根据耳廓活检结果以及三个主要症状（双侧耳廓软骨炎、炎性关节炎和眼部炎症），她的病情被诊断为RP。
通过泼尼松龙（PSL）、甲泼尼龙和环磷酰胺联合治疗，随后采用PSL、甲氨蝶呤和英夫利昔单抗联合治疗，三个主要症状逐渐改善。然而，初诊后一个月，尽管持续治疗，仍突然出现一种独特的环形周边角膜炎，角膜浸润在外周区域逐渐聚集并缩小。在减少类固醇药物剂量时，眼痛和耳痛出现加重和缓解。将全身治疗改为PSL、甲氨蝶呤和托珠单抗后，整体状况有所改善。
角膜炎在初诊后10个月内逐渐消失。
这是首例关于RP导致独特的环形周边角膜炎的病例报告。尽管使用了局部和全身类固醇药物，仍发生了这种角膜炎，且随着整体病情恢复而改善。这可能表明，对于RP患者角膜炎的恢复，稳定整体状况很重要。
