Ocular Mucous Membrane Pemphigoid: The Effect of Risk Factors at Presentation on Treatment Outcomes

Abstract

PurposeAnalyze the influence of risk factors at presentation in the long-term immunosuppressive therapy (IMT) outcomes of ocular mucous membrane pemphigoid (OMMP). DesignRetrospective multicenter study. ParticipantsOMMP patients seen at Duke Eye Center, Tecnologico de Monterrey, and Hospital Clinic of Barcelona from 1990–2022. MethodsData at presentation on demographics, direct immunofluorescence and ocular findings, extraocular sites of involvement (EOM) and prior treatments, in patients with a clinical and/or laboratory diagnosis of OMMP, were analyzed with multivariable analysis and Kaplan-Meier plots to identify factors associated with adverse outcomes. Main outcome measures(1) inflammatory control (no conjunctival inflammation in both eyes at three months on IMT); (2) relapse (new-onset inflammation after absolute control in either eye); (3) progression (≥1 cicatrizing stage progression in either eye); and (4) vision loss (>2 Snellen lines). Results117 patients (234 eyes), 61% (71/117) women, with a mean age of 66.6 (standard deviation (SD) 12.4, range: 37–97) years and a median follow-up of 34 (interquartile range Q1 16, Q3 66; range: 3–265) months. Inflammatory control was achieved in 57% (67/117) patients, with high-risk EOM (HR-EOM), including esophageal, nasopharyngeal, and/or genital [adjusted odds ratio (aOR) 12.51; 95% confidence interval (CI), 2.61–59.99; P=0.002] and corneal scarring (aOR 3.06; 95% CI, 1.15–8.14; P=0.025) as significant risk factors for persistent inflammation. Disease relapse, progression, and vision loss occurred in 20% (23/117), 12% (14/117), and 27% (32/117) of patients, respectively. Baseline corneal scarring was a risk factor for relapse [adjusted hazard ratio 4.14; 95% CI, 1.61–10.62; P=0.003], progression (aOR 11.46; 95% CI, 1.78–73.75; P=0.010), and vision loss (aOR 3.51; 95% CI, 1.35–9.10, P=0.010). HR-EOM was associated with stage progression (aOR 34.57; 95% CI, 6.57–181.89; P<0.001) and vision loss (aOR 8.42; 95% CI, 2.50–28.42, P=0.001). There were no significant differences between IMT regimes and relapse (P=0.169). ConclusionsOMMP presenting with HR-EOMs and corneal scarring has an increased risk of stage progression and vision loss. Corneal scarring and severe inflammation at baseline were associated with an increased risk of relapse. A disease progression staging system incorporating both the HR-EOMs and corneal involvement is required to predict the visual outcome of OMMP better.