利妥昔单抗治疗难治性眼瘢痕性类天疱疮1例。

IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL
Sania Vidas Pauk, Antonela Geber, Iva Bešlić, Ines Lakoš-Jukić, Tomislav Kuzman
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引用次数: 0

摘要

背景与临床意义:眼瘢痕性类天疱疮(OCP)是一种少见的影响结膜和口腔黏膜的自身免疫性疾病。慢性炎症引起结膜瘢痕,导致睑粘连、倒睫、角膜损伤,甚至可能失明。临床诊断,活检和免疫荧光支持。治疗包括全身皮质类固醇、免疫抑制剂和难治性病例的生物制剂。病例介绍:64岁男性,左眼出现眼刺激、倒睫和数指视力。裂隙灯检查显示左眼结膜炎症、角膜上皮缺损、睑粘连。活检证实眼部瘢痕性类天疱疮(OCP)。患者接受局部类固醇、环孢素、结膜下注射和全身皮质类固醇治疗,随后进行手术,BCVA改善至0.10 logMAR。两年后,疾病进展导致严重炎症和双眼视力下降。系统硫唑嘌呤和皮质类固醇可部分控制。由于反应不足,开始了利妥昔单抗治疗,导致炎症显著减少和疾病稳定。右眼BCVA改善至0.16 logMAR;患者在病情加重期间继续接受利妥昔单抗治疗,并定期随访。结论:早期诊断和及时的系统治疗是预防OCP视力丧失的关键。在难治性病例中,生物制剂如利妥昔单抗可提供有效的疾病控制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Rituximab Therapy in Refractory Ocular Cicatricial Pemphigoid: A Case Report.

Background and Clinical Significance: Ocular cicatricial pemphigoid (OCP) is a rare autoimmune disease affecting the conjunctiva and oral mucosa. Chronic inflammation causes conjunctival scarring, leading to symblepharon, trichiasis, corneal damage, and possible blindness. Diagnosis is clinical, supported by biopsy and immunofluorescence. Treatment includes systemic corticosteroids, immunosuppressants, and biologics in refractory cases. Case Presentation: A 64-year-old male presented with ocular irritation, trichiasis, and counting fingers (CF) visual acuity in the left eye. Slit-lamp examination revealed conjunctival inflammation, corneal epithelial defect, and symblepharon in the left eye. Biopsy confirmed ocular cicatricial pemphigoid (OCP). He was treated with topical steroids, cyclosporine, subconjunctival injections, and systemic corticosteroids, followed by surgery, which improved BCVA to 0.10 logMAR. Two years later, disease progression resulted in severe inflammation and visual decline in both eyes. Systemic azathioprine and corticosteroids achieved partial control. Due to insufficient response, rituximab therapy was initiated, leading to significant reduction in inflammation and stabilization of disease. Right eye BCVA improved to 0.16 logMAR; the left remained at CF. The patient continues to receive rituximab during exacerbations and is under regular follow-up. Conclusions: Early diagnosis and timely systemic treatment are essential in preventing vision loss in OCP. In refractory cases, biologic agents like rituximab may offer effective disease control.

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