title: Evaluation of early and late presentation of patients with ocular mucous membrane pemphigoid to two major tertiary referral hospitals in the United Kingdom.
abstract: OBJECTIVE
  Ocular mucous membrane pemphigoid (OcMMP) is a sight-threatening autoimmune disease in which referral to specialists units for further management is a common practise. This study aims to describe referral patterns, disease phenotype and management strategies in patients who present with either early or established disease to two large tertiary care hospitals in the United Kingdom.
  
  
  METHODS
  In all, 54 consecutive patients with a documented history of OcMMP were followed for 24 months. Two groups were defined: (i) early-onset disease (EOD:<3 years, n=26, 51 eyes) and (ii) established disease (EstD:>5 years, n=24, 48 eyes). Data were captured at first clinic visit, and at 12 and 24 months follow-up. Information regarding duration, activity and stage of disease, visual acuity (VA), therapeutic strategies and clinical outcome were analysed.
  
  
  RESULTS
  Patients with EOD were younger and had more severe conjunctival inflammation (76% of inflamed eyes) than the EstD group, who had poorer VA (26.7%=VA<3/60, P<0.01) and more advanced disease. Although 40% of patients were on existing immunosuppression, 48% required initiation or switch to more potent immunotherapy. In all, 28% (14) were referred back to the originating hospitals for continued care. Although inflammation had resolved in 78% (60/77) at 12 months, persistence of inflammation and progression did not differ between the two phenotypes. Importantly, 42% demonstrated disease progression in the absence of clinically detectable inflammation.
  
  
  CONCLUSIONS
  These data highlight that irrespective of OcMMP phenotype, initiation or escalation of potent immunosuppression is required at tertiary hospitals. Moreover, the conjunctival scarring progresses even when the eye remains clinically quiescent. Early referral to tertiary centres is recommended to optimise immunosuppression and limit long-term ocular damage.
authors.affiliation: Academic Unit of Ophthalmology, School of Immunity and Infection, University of Birmingham, Birmingham and Midland Eye Centre, Birmingham, UK.
authors.forename: G P
authors.initials: GP
authors.lastname: Williams
authors.forename: C
authors.initials: C
authors.lastname: Radford
authors.forename: P
authors.initials: P
authors.lastname: Nightingale
authors.forename: J K G
authors.initials: JK
authors.lastname: Dart
authors.forename: S
authors.initials: S
authors.lastname: Rauz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D019468:Disease Management; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D010818:Practice Patterns, Physicians'; D012017:Referral and Consultation; D006113:United Kingdom; D014792:Visual Acuity / Q000502:physiology
chemical_list: D007166:Immunosuppressive Agents