Cornea Service, The Cornea Institute, L V Prasad Eye Institute, Hyderabad, India.
Department of Rheumatology, Nizams Institute of Medical Services, Hyderabad, India.
Ocul Immunol Inflamm. 2023 May;31(4):870-873. doi: 10.1080/09273948.2022.2058555. Epub 2022 Jun 13.
To report a challenging case of relapsing polychondritis with bilateral diffuse scleritis, with 17-year follow-up.
Case report.
A 36-year-old female presented 17 years ago with bilateral diffuse scleritis and peripheral corneal infiltrates. Detailed systemic work-up was negative. Fourteen months later, she developed saddle nose deformity, debilitating myalgias, and severe recurrence of scleritis clinching the diagnosis of relapsing polychondritis. Despite high-dose oral corticosteroids, oral immunosuppressants, and cyclophosphamide infusions and adalimumab infusions, the condition showed waxing and waning over the next decade. In 2017, she was started on Tocilizumab injections after which both the systemic and ocular conditions stabilised and has been remained stable for the past 4 years.
Relapsing polychondritis has a well-known association with scleritis. The ocular disease may precede systemic symptoms in some cases. Newer agent such as tocilizumab appears to be effective in controlling this relentless and recurrent disease.
报告一例复发性多软骨炎伴双侧弥漫性巩膜炎的挑战性病例，随访 17 年。
病例报告。
一名 36 岁女性 17 年前出现双侧弥漫性巩膜炎和周边角膜浸润。详细的系统检查为阴性。14 个月后，她出现鞍鼻畸形、进行性肌痛和严重的巩膜炎复发，确诊为复发性多软骨炎。尽管使用了大剂量口服皮质类固醇、口服免疫抑制剂、环磷酰胺输注和阿达木单抗输注，但在接下来的十年中病情呈时好时坏的趋势。2017 年，她开始接受托珠单抗注射治疗，此后全身和眼部状况均稳定，并在过去 4 年中保持稳定。
复发性多软骨炎与巩膜炎有明确的相关性。在某些情况下，眼部疾病可能先于全身症状出现。新型药物如托珠单抗似乎能有效控制这种顽固且反复发作的疾病。
