Objectives: Mucous membrane pemphigoid with ocular involvement (MMPO) is a sight-threatening autoimmune disease that may lead to severe conjunctival cicatrization and keratopathy. The peak age of onset is in the seventh decade, although the disease may also occur in younger patients (< 60 years). This study was designed to evaluate the clinical features of young patients with MMPO and to assess the clinical outcome when compared with patients in the > 70 age group.
Design: Retrospective, comparative, interventional case series.
Participants: Eighteen patients under the age of 60 years and 18 patients above the age of 70.
Methods: Patients with documented MMPO were identified from the External Diseases Immunosuppression Database.
Main outcome measures: Stage of disease (Foster, Mondino), visual acuity, and ocular complications (lid, conjunctival, corneal) were evaluated at presentation, the time when immunosuppression was commenced, and final follow-up. Disease progression, control of ocular inflammation with systemic immunosuppression, the incidence of mucocutaneous lesions, and surgical intervention were also assessed.
Results: Patients in the 2 groups (young and classic age groups) were observed for 61 (range, 29-218) and 69 (range, 12-193) months, respectively (P = 0.94). Median ages at the start of immunosuppression were 48.7 (range, 29-60) and 77.6 (range, 71-85) years. Mucocutaneous involvement was more common in the young than in the classic age group (13 [72%], 7 [39%]; P < 0.05). Ocular staging (Mondino, Foster) at presentation, the start of immunosuppression, and final follow-up was more advanced in the younger patients. There was no statistical difference in visual acuity, individual ocular complications, or incidence of surgical intervention between the 2 groups throughout the course of the study.
Conclusion: Younger patients with MMPO present with more severe ocular and systemic disease and, despite immunosuppression, progress more rapidly.