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

被引:44
作者
Georgoudis, Panagiotis [1 ]
Sabatino, Francesco [2 ]
Szentmary, Nora [3 ,4 ]
Palioura, Sotiria [5 ]
Fodor, Eszter [4 ]
Hamada, Samer [6 ]
Scholl, Hendrik P. N. [7 ,8 ]
Gatzioufas, Zisis [7 ]
机构
[1] Barts Hlth NHS Trust, Whipps Cross Univ Hosp, London, England
[2] Moorfields Eye Hosp, London, England
[3] Saarland Univ, Med Ctr, Dept Ophthalmol, Homburg, Germany
[4] Semmelweis Univ, Dept Ophthalmol, Budapest, Hungary
[5] Athens Vis Eye Inst, Athens, Greece
[6] Queen Victoria Hosp, Corneoplast Unit, E Grinstead, England
[7] Basel Univ Hosp, Dept Ophthalmol, Basel, Switzerland
[8] Inst Mol & Clin Ophthalmol Basel IOB, Basel, Switzerland
基金
英国惠康基金;
关键词
Diagnostics; Mucous; Ocular pemphigoid; Pathophysiology; Treatment; SYNDROME/TOXIC EPIDERMAL NECROLYSIS; CICATRISING CONJUNCTIVITIS; FORNIX DEPTH; SYSTEMIC CHEMOTHERAPY; MEDICAL-TREATMENT; BETA-4; INTEGRIN; LID ENTROPION; INVOLVEMENT; THERAPY; PATHOGENESIS;
D O I
10.1007/s40123-019-0164-z
中图分类号
R77 [眼科学];
学科分类号
100212 ;
摘要
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.
引用
收藏
页码:5 / 17
页数:13
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