July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Disease Relapse after Drug-Free Remission in Mucous Membrane Pemphigoid
Author Affiliations & Notes
  • Amde Selassie Shifera
    Ophthalmology, Johns Hopkins University, Baltimore, Maryland, United States
  • Gloria H Hong
    Johns Hopkins University, Maryland, United States
  • Irfan Khan
    University of Virginia, Virginia, United States
  • Chinwenwa Okeagu
    NIH, Maryland, United States
  • Jennifer E Thorne
    Ophthalmology, Johns Hopkins University, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Amde Selassie Shifera, Gilead (F); Gloria Hong, None; Irfan Khan, None; Chinwenwa Okeagu, None; Jennifer Thorne, None
  • Footnotes
    Support  King Khaled Eye Specialist Hospital; Gilead; Research to Prevent Blindness
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 6707. doi:
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    • Get Citation

      Amde Selassie Shifera, Gloria H Hong, Irfan Khan, Chinwenwa Okeagu, Jennifer E Thorne; Disease Relapse after Drug-Free Remission in Mucous Membrane Pemphigoid. Invest. Ophthalmol. Vis. Sci. 2019;60(9):6707.

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

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Purpose : To quantitate the risk of relapse of ocular and extraocular disease among patients with mucous membrane pemphigoid (MMP) who have undergone drug-free remission.

Methods : 61 of 93 patients treated for biopsy-proven MMP in the JHMI DOI database underwent drug-free remission, defined as no evidence of active inflammation or progression of conjunctival cicatrization and on no systemic medication for MMP for 3 months or longer. Primary outcomes measures were the recurrence of ocular MMP or active MMP in the nose, mouth, pharynx or skin. Incidence rates and median times to relapse for ocular and extra-ocular disease were calculated using Kaplan-Meier methodology.

Results : Over a median follow-up time of 7 years (range=1-22 years), 61 of 93 patients (66%) experienced drug-free remission. The majority of these patients (47/61, 77%) received cyclophosphamide therapy. Eleven of the 61 patients (18%) had disease relapse after remission: 4 with ocular disease only, 3 with extra-ocular disease only and 4 with both. The median time from remission to relapse of ocular MMP was 30 months (rate=0.032/person-year [PY], 95% confidence interval [CI]: 0.012/PY to 0.077/PY), and the median time to relapse of MMP at any site (ocular or extra-ocular site) was 25 months (rate=0.043/PY, 95% CI: 0.025/PY to 0.067/PY). Use of cyclophosphamide was associated with a lower risk of relapse (hazard ratio=0.49, P=0.05) compared to other immunosuppressive drugs except for rituximab, but the statistical significance was marginal. Five patients experienced drug-free remission after rituximab therapy and none of them had relapse, but the duration of follow-up in these patients was low.

Conclusions : Rates of relapse of MMP after drug-free remission are low but not zero; therefore monitoring of patients remains necessary. Relapses were not observed among those patients treated with rituximab who had remission; however follow-up in these patients is limited and the sample size is small. Additional research regarding treatment outcomes with rituximab are needed.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.


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