Clinical Characteristics and Outcomes of Ocular Cicatricial Pemphigoid: A Cohort Study and Literature Review

Abstract

Purpose: Ocular cicatricial pemphigoid (OCP) is a rare systemic autoimmune disease and a potentially blinding subepithelial blistering disorder. The purpose of this study was to describe the clinical spectrum of the disease and to assess the efficacy and safety of immunosuppressive agents in a cohort of patients with OCP. Methods: We conducted a monocentric retrospective cross-sectional cohort study of all unselected consecutive patients diagnosed with progressive OCP. Ocular and extra ophthalmological involvement as well as histological findings were gathered. Other outcomes were exposures to immunosuppressive agents defined by the use of a particular treatment. For each exposure, success in controlling ocular inflammation was graded as a complete response, response, or failure. Relapses and adverse events (AE) were also recorded. Results: Seventeen patients (34 affected eyes), 35% of whom were women, were included, with an age at diagnosis of 75 ± 11 years. Corneal involvement was diagnosed in 30 of 34 eyes, and 22 of 34 eyes had progressive fibrosing conjunctival involvement. Sixty-two exposures to immunosuppressive agents or biologics were recorded: dapsone, n = 26; mycophenolate mofetil, n = 6; azathioprine, n = 4; cyclophosphamide, n = 10; rituximab, n = 14; and intravenous immunoglobulin, n = 2. Rates of response and of complete response achievement during the first 3 months were 84% and 45%, respectively. Response rates were 100%, 100%, 86%, 85%, and 80% for intravenous immunoglobulin, mycophenolate mofetil, rituximab, dapsone, and cyclophosphamide, respectively. Thirteen percent of those drugs were discontinued because of an adverse event in 4 patients. Conclusions: This study describes the efficacy of immunosuppressants or biologics with an acceptable safety profile for OCP.

The progression and therapeutic outcome of ocular mucous membrane pemphigoid in a Mexican-mestizo population

“…After three months on IMT, 59% of the eyes achieved a complete response. Fremont et al reported a signi cantly lower complete response rate (32%) to the rst line immunosuppressants [4]. Our hypothesis to the hindmost are two-fold.…”

“…A partial response was de ned as a reduction of in ammation by at least one grade, and no response as persistent in ammation. This approach has been previously used by other authors [4,14]. Therapy adverse events were de ned as side effects requiring drug discontinuation, whereas disease recurrence was de ned as new-onset conjunctival in ammation after achieving a complete response.…”

“…This disorder belongs to the spectrum of diseases known as mucous membrane pemphigoid (MMP), and typically affects genetically predisposed mature women, but could affect both sexes at an earlier age [2,3]. OMMP is considered a rare disorder, with an incidence ranging from 1:12,000 to 1:60,000 person-years [3,4]. However, it is the most common cause of cicatrizing conjunctivitis in developed countries [5].…”

Corneal perforation in ocular cicatricial pemphigoid

“…For the aggressive forms of OCP or treatment failure groups, Rituximab should be considered. [ 8 , 9 ] Despite the effectiveness of systemic immunosuppressant, discontinuation rates vary and poor compliance were still noted in some cases. [ 8 ] In addition, OCP patients are general elderly with an advanced stage by the time of diagnosis.…”

“…Although it seems to be sufficient to stop disease evolution, no conclusion can be drawn on the efficacy of topical anti-inflammatory treatment. [ 9 , 10 ] In our patient, systemic immunosuppressive therapy was not used during the perioperative period due to the potential side effects and drug toxicity. Furthermore, we believed that topical ciclosporin and topical prednisolone aid in alleviating the conjunctival inflammation and shrinkage.…”