Managing Ocular Cicatricial Pemphigoid
BY WILLIAM L. MILLER, OD, PHD, FAAO
Mucous membrane pemphigoid is an autoimmune disease that predominantly affects the body’s mucous membranes, with the eye being the second most commonly affected site. Ocular cicatricial pemphigoid (OCP) is an uncommon yet potentially devastating disease affecting vision. Rates of OCP range between 1 in 12,000 to 1 in 60,000, with an average age of incidence of 65 years (Foster, 1986 and 2011).
Signs and Symptoms
OCP symptoms are nonspecific and precede diagnosis by an average of 2.8 years (Foster, 1986). General clinical signs include blistering lesions with resultant scarring of the mucous membranes. Early signs may begin as unilateral, recurrent cases of papillary conjunctivitis. Consider OCP when no infection is evident or when other causes are not clearly discernible.
Other manifestations of OCP include conjunctival fibrosis and conjunctival shortening, which may also cause eyelid abnormalities such as trichiasis, entropion, and lagophthalmos. An impaired ocular surface will lead to dry eye late in the disease process. The mucosal membrane will shrink and both the cornea and conjunctiva will scar as OCP progresses. Its final stages will result in keratinization of the cornea.
A histopathologic evaluation using immunofluorescent and immunoperoxidase testing after a conjunctival biopsy can confirm the diagnosis of OCP (Tauber et al, 1991). Grading is incumbent on the level of conjunctival forniceal shortening (Mondino, 1990).
Managing OCP patients involves a multidisciplinary approach coordinated among the involved physicians to manage the multiple body systems affected. The treatment goals are to decrease inflammation and to prevent the cicatricial effects of the disease.
Ocular treatment may range from topical anti-inflammatories to surgery. Nonpreserved tear supplements and/or punctal occlusion can help with dry eye complaints. Blepharoconjunctivitis can be managed with warm compresses and topical antibiotics, while eyelid abnormalities usually require surgery.
Medical therapy can include oral corticosteroids, alkylating agents, antimetabolites, immunotherapy, and intravenous immunoglobulin. Oral corticosteroids are typically used as a bridge therapy until the immunomodulatory medications reach sufficient therapeutic levels.
Srikumaran and Akpek (2012) suggest a stepped approach based on disease severity. Kirzhner and Jakobiec (2011) report improvement within one month using diaminodiphenyl-sulfone for mild-to-moderate cases. Contraindications to this treatment include G6PD deficiencies and sulfa allergies. Patients in this group can be started on mycophenolate mofetil or methotrexate.
More rapid and severe forms of OCP are treated with oral steroids and cyclophosphamide. Etanercept and infliximab are also used in cases that are refractory to corticosteroid and first line immunosuppressive therapy. Foster et al (2010) reported the effectiveness of using rituximab and intravenous immunoglobulin.
Best to Catch it Early
Even with timely therapy and lack of evident inflammation, 30 percent to 42 percent of patients will continue to progressively worsen (Williams et al, 2011). Even so, early OCP detection and treatment can prolong vision and improve quality of life. CLS
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Dr. Miller is an associate professor and chair of the Clinical Sciences Department at the University of Houston College of Optometry. He is a consultant or advisor to Alcon and Vistakon and has received research funding from Alcon and CooperVision and lecture or authorship honoraria from Alcon and B+L. You can reach him at email@example.com.