Caiyun You1,2, Lina Ma1,3, Stephen D Anesi1,3, C Stephen Foster1,3,4. 1. 1 Massachusetts Eye Research and Surgery Institution (MERSI), Waltham, MA - USA. 2. 3 Department of Ophthalmology, Tianjin Medical University General Hospital, Tianjin - China. 3. 2 Ocular Immunology and Uveitis Foundation, Weston, MA - USA. 4. 4 Department of Ophthalmology, Harvard Medical School, Boston, MA - USA.
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 Long-term remission off IMT for OCP was defined as patients withdrawn from IMT ≥1 year lacking clinically detectable progressive scarring according to Foster staging and subjective assessment.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 CONCLUSIONS: Long-term remission for OCP off IMT may be achieved after stepladder IMT is implemented and withdrawn.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.
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 Long-term remission off IMT for OCP was defined as patients withdrawn from IMT ≥1 year lacking clinically detectable progressive scarring according to Foster staging and subjective assessment.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.
Authors: E Schmidt; H Rashid; A V Marzano; A Lamberts; G Di Zenzo; G F H Diercks; S Alberti-Violetti; R J Barry; L Borradori; M Caproni; B Carey; M Carrozzo; G Cianchini; A Corrà; F G Dikkers; C Feliciani; G Geerling; G Genovese; M Hertl; P Joly; J M Meijer; V Mercadante; D F Murrell; M Ormond; H H Pas; A Patsatsi; S Rauz; B D van Rhijn; M Roth; J Setterfield; D Zillikens; G Zambruno; B Horváth; F Caux Journal: J Eur Acad Dermatol Venereol Date: 2021-07-26 Impact factor: 6.166