Rheumatology Department, County Emergency Clinical Hospital Cluj, Centre for Rare Musculoskeletal Autoimmune and Autoinflammatory Diseases, Cluj-Napoca, Romania.
Rheumatology Department, County Emergency Clinical Hospital Cluj, Centre for Rare Musculoskeletal Autoimmune and Autoinflammatory Diseases, Cluj-Napoca, and Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):86-92. doi: 10.55563/clinexprheumatol/27n7im. Epub 2022 Feb 24.
Relapsing polychondritis (RP) evolves with variable and intermittent involvement of cartilage and proteoglycan-rich structures. Ocular manifestations are present in up to two-thirds of RP patients. Necrotising scleritis (NS) and peripheral ulcerative keratitis (PUK) may be inaugural and may lead to eye perforation and vision loss. We aimed to review NS and PUK in RP, in order to characterise them, to identify successful treatment options and unmet needs.
A systematic review of the currently available evidence in PubMed, EMBASE and Scopus was performed according to PRISMA, including observational studies, single case reports and case series of NS/PUK in RP. Study design, number of patients, age, gender, treatment and outcome, were extracted. Two RP patients also provided their opinion.
Five case reports and two case series were eligible for inclusion. We identified 10 RP patients with eye-threatening complications (NS and/or PUK), 9 adults (2 males, 7 females, aged 35-72, median age 57.6 years) and one paediatric patient (F, 11 years). Apart from glucocorticoids, cyclophosphamide was effective in 4 patients; infliximab, high-dose immunoglobulins, dapsone, or cyclosporine were also successfully employed in a case each. Surgical repair was reported in 2 cases.
Ocular inflammation is often bilateral and recurring in RP; NS/PUK are rare complications. All patients who develop NS/PUK should be specifically questioned for RP signs and symptoms. Early institution of immunosuppressive therapies is mandatory. Increasing awareness, physicians' and patients' education and a multidisciplinary approach may help improve the prognosis of these serious complications of RP.
复发性多软骨炎（RP）的特征为软骨和富含蛋白聚糖的结构出现多变且间歇性的受累。多达三分之二的 RP 患者存在眼部表现。坏死性巩膜炎（NS）和边缘溃疡性角膜炎（PUK）可能是首发表现，并可导致眼球穿孔和视力丧失。我们旨在综述 RP 中的 NS 和 PUK，以对其进行特征描述，确定有效的治疗选择和未满足的需求。
根据 PRISMA 标准，对 PubMed、EMBASE 和 Scopus 中目前可用的证据进行了系统综述，纳入了 NS/PUK 与 RP 的观察性研究、单病例报告和病例系列研究。提取研究设计、患者数量、年龄、性别、治疗和结局。两位 RP 患者也提供了他们的意见。
有 5 份病例报告和 2 份病例系列研究符合纳入标准。我们共确定了 10 例出现眼部威胁性并发症（NS 和/或 PUK）的 RP 患者，包括 9 例成人（2 例男性，7 例女性；年龄 35-72 岁，中位年龄 57.6 岁）和 1 例儿童（女性，11 岁）。除了糖皮质激素外，环磷酰胺对 4 例患者有效；英夫利昔单抗、高剂量免疫球蛋白、氨苯砜或环孢素也分别在 1 例患者中成功使用。有 2 例患者接受了手术修复。
眼部炎症在 RP 中常为双侧且反复发作；NS/PUK 是罕见的并发症。所有出现 NS/PUK 的患者均应特别询问是否存在 RP 症状和体征。必须尽早开始免疫抑制治疗。提高认识、对医生和患者进行教育以及多学科方法可能有助于改善这些严重的 RP 并发症的预后。
