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Long-term remission of ocular cicatricial pemphigoid off immunomodulatory therapy

Abstract

Purpose

To evaluate whether long-term remission of ocular cicatricial pemphigoid (OCP) after withdrawal of immunomodulatory therapy (IMT) is possible.

Methods

A total of 34 of 464 presenting patients (66 eyes) with biopsy-proven OCP in long-term remission off IMT were identified after finishing a 2-year IMT regimen without active disease (2005-2015). Long-term remission off IMT for OCP was defined as patients withdrawn from IMT ≥1 year lacking clinically detectable progressive scarring according to Foster staging and subjective assessment.

Results

All 34 patients achieved ≥1 year of clinical remission without IMT following 2 years IMT lacking active disease. Mean onset age of OCP was 67.0 years, and median follow-up time was 63.4 months. Mean duration between OCP onset and IMT initiation was 29.5 months, with a mean sustained remission time of 36.0 months off IMT. The mean duration of IMT prior to remission off IMT was 34.8 months (median 32 months, IQR 27-39.5 months). Commonly, methotrexate was used prior to OCP remission (19 patients; 55.9%). Two patients experienced mild flare-up postremission off IMT at months 25 and 37 and a course of topical steroid appeared to resolve the inflammation. Another patient had active inflammation at last office visit 5 years after discontinuation of IMT and will restart IMT.

Conclusions

Long-term remission for OCP off IMT may be achieved after stepladder IMT is implemented and withdrawn. Longer follow-up and more sensitive measures of scarring and inflammation are needed to generate a consensus on the definition of complete remission and on cessation of systemic IMT for OCP.

Post author correction

Article Type: ORIGINAL RESEARCH ARTICLE

Article Subject: Conjunctivitis

DOI:10.5301/ejo.5001050

Authors

Caiyun You, Lina Ma, Stephen D. Anesi, C. Stephen Foster

Article History

Disclosures

Financial support: No financial support was received for this submission.
Conflict of interest: Dr. C. Stephen Foster declares the following: consultancies with Aldeyra Therapeutics (Lexington, MA), Bausch & Lomb Surgical, Inc. (Rancho Cucamonga, CA), Eyegate Pharma (Waltham, MA), Genentech (South San Francisco, CA), Novartis (Cambridge, MA), pSivida (Watertown, MA); grants or grants pending with Aciont (Salt Lake City, UT), Alcon (Aliso Viejo, CA), Aldeyra Therapeutics (Lexington, MA), Bausch & Lomb (Bridgewater, NJ), Clearside Biomedical (Alpharetta, GA), Dompé pharmaceutical (Milan, Italy), Eyegate Pharma (Waltham, MA), Mallinckrodt Pharmaceuticals (Dublin, Ireland), Novartis Pharmaceuticals (Cambridge, MA), pSivida (Watertown, MA), Santen (Osaka, Japan); payment for lectures including service on speaking bureaus: Alcon (Aliso Viejo, CA), Allergan (Dublin, Ireland), Mallinckrodt Pharmaceuticals (Dublin, Ireland); stock or stock options: Eyegate Pharma (Waltham, MA).

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Authors

Affiliations

  • Massachusetts Eye Research and Surgery Institution (MERSI), Waltham, MA - USA
  • Ocular Immunology and Uveitis Foundation, Weston, MA - USA
  • Department of Ophthalmology, Tianjin Medical University General Hospital, Tianjin - China
  • Department of Ophthalmology, Harvard Medical School, Boston, MA - USA

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