眼瘢痕性类天疱疮机制及治疗研究进展

Progress on pathogenesis and treatment of ocular cicatricial pemphigoid

摘要:

眼瘢痕性类天疱疮(ocular cicatricial pemphigoid，OCP)是黏膜类天疱疮(mucous membrane pemphigoid，MMP)在眼部的一种特殊表现形式，目前发病机制尚不明确，可由抗原抗体反应、炎细胞浸润、细胞因子作用、钙离子水平升高、易感基因等多种因素导致。病变早期表现为慢性进行性的结膜纤维化性炎症，晚期可见角膜混浊及新生血管形成，最终导致视力严重丧失。因此，及时对OCP患者进行规范的临床治疗尤为重要。例如氨苯砜、免疫球蛋白(intravenous immunoglobulin，IVIG)、利妥昔单抗(rituximab，RTX)、肿瘤坏死因子拮抗剂、肾上腺皮质激素类药物，可以有效控制炎症反应、延缓疾病进展。手术治疗可在OCP患者并发严重倒睫、角膜病变及白内障时酌情考虑。

Abstract:

Ocular cicatricial pemphigoid(OCP)is a special manifestation of mucosal pemphigoid(mucous membrane pemphigoid, MMP), and the pathogenesis is not clear at present. It can be caused by variety factors such as antigen-antibody reaction, inflammation cell infiltration, the action of various cytokines, elevated calcium ion levels and susceptibility genes. In the early period of diseases, conjunctival present chronic progressive fibrotic inflammation, later the corneal opacity and the neovascularization will eventually lose vision. Therefore, it is particularly important to carry out clinical standardized treatment for OCP patients in a timely manner. To use medicine to control the inflammation and delay the progression of the disease, for example, dapsone, intravenous immunoglobulin(IVIG), rituximab(RTX), tumor necrosis factor antagonists and adrenocorticoids. Surgical treatment can be considered appropriately when OCP patients are complicated by severe trichiasis, corneal disease and cataract.