Evaluation of mucous membrane pemphigoid with ocular involvement in young patients.
OBJECTIVE
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.


METHODS
Retrospective, comparative, interventional case series.


METHODS
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.


METHODS
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.


CONCLUSIONS
Younger patients with MMPO present with more severe ocular and systemic disease and, despite immunosuppression, progress more rapidly.
Rauz
Saaeha
S
Corneal and External Diseases Service, Moorfields Eye Hospital, National Health Service Trust, London, United Kingdom. s.rauz@bham.ac.uk
Maddison
Patricia G
PG
Dart
John K G
JK
D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy; D003229:Conjunctival Diseases / Q000175:diagnosis* / Q000188:drug therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000188:drug therapy; D012189:Retrospective Studies; D014792:Visual Acuity
D007166:Immunosuppressive Agents