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眼部黏膜类天疱疮:病理生理学、诊断与治疗的现状

Ocular Mucous Membrane Pemphigoid: Current State of Pathophysiology, Diagnostics and Treatment.

作者信息

Georgoudis Panagiotis, Sabatino Francesco, Szentmary Nora, Palioura Sotiria, Fodor Eszter, Hamada Samer, Scholl Hendrik P N, Gatzioufas Zisis

机构信息

Whipps Cross University Hospital, Barts Health NHS Trust, London, UK.

Moorfields Eye Hospital, London, UK.

出版信息

Ophthalmol Ther. 2019 Mar;8(1):5-17. doi: 10.1007/s40123-019-0164-z. Epub 2019 Jan 29.

Abstract

Mucous membrane pemphigoid (MMP) is a systemic cicatrizing autoimmune disease that primarily affects orificial mucous membranes, such as the conjunctiva, the nasal cavity, the oropharynx, and the genitalia. Ocular involvement occurs in about 70% of all MMP cases. Ocular MMP (OcMMP) also encompasses the conditions linear immunoglobulin A disease, mucosal dominated epidermolysis bullosa acquisita, and anti-laminin 332/anti-epiligrin/anti-laminin 5 pemphigoid. It is a complex clinical entity that may lead to ocular surface failure and result in inflammatory and infectious complications, as well as potentially devastating visual loss. Early diagnosis and appropriate treatment are of paramount importance and require a high level of expertise as this condition can be extremely challenging to diagnose and treat even for experienced clinicians. In this review we provide an up-to-date insight on the pathophysiology of OcMMP, with an emphasis on the current state of its diagnostics and therapeutics. Our the aim is to increase our understanding of OcMMP and highlight modern diagnostic and therapeutic options.

摘要

黏膜类天疱疮(MMP)是一种全身性瘢痕性自身免疫性疾病,主要累及口腔黏膜,如结膜、鼻腔、口咽和生殖器。约70%的MMP病例会出现眼部受累。眼部黏膜类天疱疮(OcMMP)还包括线性免疫球蛋白A病、黏膜为主型获得性大疱性表皮松解症以及抗层粘连蛋白332/抗上皮层黏连蛋白/抗层粘连蛋白5类天疱疮。它是一种复杂的临床病症,可能导致眼表功能障碍,并引发炎症和感染并发症,以及潜在的严重视力丧失。早期诊断和恰当治疗至关重要,且需要高水平的专业知识,因为即使对于经验丰富的临床医生而言,这种病症的诊断和治疗也极具挑战性。在本综述中,我们提供了关于OcMMP病理生理学的最新见解,重点关注其诊断和治疗的现状。我们的目的是增进对OcMMP的理解,并突出现代诊断和治疗选择。

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