眼瘢痕性类天疱疮的治疗策略

Therapeutic strategies for ocular cicatricial pemphigoid

眼瘢痕性类天疱疮(OCP)是一种罕见的累及眼结膜的自身免疫病,常由于其早期的非特异性临床表现导致误诊或延误治疗,最终造成眼表结构功能损害及失明。OCP治疗主要通过全身免疫抑制控制炎症反应及瘢痕进展,经过多年的临床探索及众多新药问世,目前治疗期前景乐观。现全身免疫抑制治疗主要包括传统免疫抑制治疗(CIST)及新型免疫抑制治疗。CIST现主要为治疗OCP的一线用药,但由于其副作用较多、对顽固性OCP疗效不佳而有一定缺陷;新型免疫抑制药物如IVIg、抗TNF-α药及单克隆抗体等,凭借强力的免疫抑制作用及较少副反应逐渐受到重视,在今后可能可作为一线用药的更优选择。中晚期OCP患者因并发眼表畸形及角膜浑浊等严重影响生活质量,在全身炎症情况控制稳定的前提下可行手术治疗。局部治疗主要针对不同的眼表并发症行相应处理,以改善局部症状。经过科学合理的药物及手术治疗,大部分OCP患者预后良好,但仍有少量反复发作者需进一步研究,寻找最为合适的治疗策略。

Ocular cicatricial pemphigoid（ OCP） is a rare autoimmune disease that affects conjunctiva. Because of its nonspecific manifestations in early stages,OCP usually gets neglected or misdiagnosed and can lead to permanent ocular surface dysfunction or even blindness. The therapeutic strategies for OCP mainly consist of two mainstreams： systemic immunosuppression and surgery. Conventional systemic immunosuppressive therapy（ CIST） is first line choice despite of many of its adverse effects and possibility of resistance. In contrast,novel immunotherapeutic Methods have stronger immunosuppressive effects with less adverse effects,and may become a better choice in the future. For late-stage OCP patients who suffer from ocular surface dysfunction and cornea opacification,surgery may help improve their quality of life as long as the inflammation has been controlled properly. Most OCP patients have favorable prognosis with proper treatment. However,a small portion of OCP patients will have recurrent inflammation,which still needs further studies to find an effective solution.