IRCCS - Fondazione Bietti, Rome, Italy.
Department of Sensory Organs, "Sapienza" University of Rome, V. le del Policlinico 155, 00161, Rome, Italy.
Immunol Res. 2019 Jun;67(2-3):280-289. doi: 10.1007/s12026-019-09087-7.
Ocular mucous membrane pemphigoid (MMP) is a rare, immuno-mediated chronic progressive condition of the conjunctiva characterized by blisters developing from sub-epithelial tissue through disruption of the adhesions between the conjunctival epithelium and the sub-epithelium. Patients with ocular MMP, in many cases, develop profound conjunctival scarring and visual impairment. Furthermore, ocular MMP may lead to a progressive secondary corneal vascularization and to corneal opacification. Ocular MMP is difficult to diagnose during the initial stages because of false negatives during biopsy and variability in the clinical presentation. Most of the current pharmacological treatments aim to control the inflammatory response to reduce the progressive tissue remodeling which leads to the formation of a fibrotic scar. The course and prognosis of ocular MMP depend on the severity and progression of the disease after systemic immunomodulatory therapy. The aim of this review is to provide a comprehensive analysis of the current literature on established and emerging concepts in ocular MMP, with special attention to its clinical presentation, diagnosis, treatment, and pathogenic mechanisms, including the role of some cytokines and growth factors in the development of the disease.
眼黏膜类天疱疮(MMP)是一种罕见的、免疫介导的慢性进行性结膜疾病,其特征是通过破坏结膜上皮和下皮之间的粘连,在下皮组织中形成水疱。在许多情况下,患有眼 MMP 的患者会出现严重的结膜瘢痕和视力损害。此外,眼 MMP 可能导致进行性继发性角膜血管化和角膜混浊。由于活检时存在假阴性和临床表现的可变性,眼 MMP 在初始阶段很难诊断。目前大多数药物治疗的目的是控制炎症反应,以减少导致纤维性瘢痕形成的进行性组织重塑。眼 MMP 的病程和预后取决于全身免疫调节治疗后疾病的严重程度和进展情况。本文的目的是对眼 MMP 的现有和新兴概念的文献进行全面分析,特别关注其临床表现、诊断、治疗和发病机制,包括一些细胞因子和生长因子在疾病发展中的作用。