June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Ocular Involvement in Mucous Membrane Pemphigoid
Author Affiliations & Notes
  • Gloria H. Hong
    Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Irfan Khan
    Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Amde Shifera
    Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Chinwenwa Okeagu
    Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Jennifer E Thorne
    Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Gloria Hong, None; Irfan Khan, None; Amde Shifera, None; Chinwenwa Okeagu, None; Jennifer Thorne, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 561. doi:
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    • Get Citation

      Gloria H. Hong, Irfan Khan, Amde Shifera, Chinwenwa Okeagu, Jennifer E Thorne; Ocular Involvement in Mucous Membrane Pemphigoid. Invest. Ophthalmol. Vis. Sci. 2017;58(8):561.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Mucous membrane pemphigoid (MMP) is a potentially life threatening, autoimmune disease affecting various mucosal surfaces including the conjunctiva. Ocular MMP can lead to blindness if untreated. The purpose of this study is to describe the demographic and clinical features of MMP patients with and without ocular disease at presentation; to calculate the risk of developing ocular MMP or new extra-ocular site of MMP during follow up; and to identify risk factors for new-onset disease.

Methods : We performed a retrospective chart review of 162 biopsy-proven MMP patients. Wilcoxon rank-sum test and chi-square test were used to compare characteristics of those with and without ocular MMP. Kaplan-Meier curves were used to describe incidence of ocular MMP and development of a secondary extra-ocular location of activity. Cox regression models were used to identify risk factors for new-onset ocular MMP and new extra-ocular site of MMP.

Results : At presentation, 109 of 162 MMP patients (67.3%) had ocular involvement. The median [interquartile range, IQR] age was higher for ocular MMP patients than those without ocular disease (68 [59, 78] years vs. 61 [49, 69] years, p=0.004). Patients with ocular involvement were more likely to be male (49.5% vs. 30.2%, p=0.02). A history of trichiasis was more common among patients with ocular MMP (60.6% vs. 5.7%, p<0.001). At presentation, 78 (71.6%) of patients with ocular MMP also had extra-ocular involvement, with the oropharynx (89.7%) most commonly involved site. Over a median [IQR] follow-up time of 7.1 [2.6, 12.5] years (range=3 months to 22 years), the risk of developing ocular MMP was 0.014 per eye-year (EY) (95% confidence interval [CI]: 0.005/EY, 0.034/EY). The risk of developing a new location of extra-ocular MMP was 0.020/EY (95% CI: 0.007/EY, 0.043/EY). Smoking was a risk factor for developing an additional extra-ocular MMP location (hazard ratio=4.09, p=0.05).

Conclusions : MMP patients with ocular involvement were more likely to be older, male, and have a history of trichiasis than those without ocular involvement. Patients presenting with only extra-ocular MMP are at risk for developing ocular MMP, and all MMP patients are at risk for developing secondary extra-ocular MMP locations, although these risks are low. Long-term follow-up is necessary to detect new-onset disease.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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