Ocular cicatricial pemphigoid:diagnosis and systemic management  

作　　者：Claudia Castiblanco 

机构地区：[1]Department of Ophthalmology and Visual Sciences,Albert Einstein College of Medicine,Montefiore Medical Center,Bronx,NY,USA

出　　处：《Annals of Eye Science》2024年第2期38-52,共15页眼科学年鉴（英文）

摘　　要：Ocular cicatricial pemphigoid(OCP)is a subcategory of mucous membrane pemphigoid(MMP)where the conjunctiva is the main site of inflammation.It is a chronic and autoimmune disease characterized by acute and chronic conjunctivitis that can progress to severe conjunctival cicatrization,corneal opacification,ocular surface keratinization,and eyelid abnormalities.OCP can lead to structural damage that can result in visual impairment,visual loss,and blindness,and can have a significant impact in a patient’s quality of life.Patients may manifest with varying symptoms,degrees of severity and may have different rates of progression.Early diagnosis and appropriate systemic immunosuppression are of utmost importance for prompt and adequate disease control.Various systemic immunomodulatory therapies(IMTs),including anti-metabolites,alkylating,and biologic agents have been utilized to achieve inflammation control and remission.Careful monitoring of disease progression is important to assess response and to modify and escalate therapy if needed.Treatment to alleviate symptoms of dry eye disease and address trichiasis and other eyelid abnormalities is recommended as well.A multidisciplinary approach to optimize clinical care is recommended in the management of patients with OCP.This review will address the immunopathogenesis,clinical features,keys to diagnosis and staging of patients with OCP.It will highlight the current immunomodulators utilized for disease management and proposed stepladder strategies.This review will discuss the updated roles of combination therapy,novel use of biologics as well as the recent use of adrenocorticotropic hormone(ACTH)analog in severe recalcitrant cases.

关 键 词：Ocular cicatricial pemphigoid(OCP) mucous membrane pemphigoid(MMP) basement membrane zone(BMZ) symblepharon immunomodulatory therapy(IMT) 

分 类 号：R771.3[医药卫生—眼科] R593.2[医药卫生—临床医学]