Author:
Bohelay Gérôme,Alexandre Marina,Le Roux-Villet Christelle,Sitbon Ishaï,Doan Serge,Soued Isaac,Shourick Jason,Rousset Laurie,Mellottee Benoît,Heller Michel,Lièvre Nicole,Zumelzu Coralie,Morin Florence,Grootenboer-Mignot Sabine,Gabison Eric,Caux Frédéric,Prost-Squarcioni Catherine,Musette Philippe
Abstract
Mucous membrane pemphigoid (MMP) is a heterogeneous group of rare, chronic, subepithelial autoimmune blistering diseases (AIBDs) with predominant involvement of mucous membranes that can be sight-threatening and life-threatening. Rituximab (RTX) has demonstrated its efficacy in severe MMP refractory to conventional immunosuppressants in small series that differed in RTX scheme, concomitant therapies, and outcome definitions. In a meta-analysis involving 112 patients with MMP treated with RTX, complete remission (CR) was reported in 70.5% of cases. Herein, we report the largest retrospective monocentric study on RTX efficacy in a series of 109 severe and/or refractory patients with MMP treated with RTX with a median follow-up period of 51.4 months. RTX was administered in association with immunomodulatory drugs (dapsone, salazopyrine) without any other systemic immunosuppressant in 104 patients. The RTX schedule comprised two injections (1 g, 2 weeks apart), repeated every 6 months until CR or failure, with a unique consolidation injection (1 g) after CR. The median survival times to disease control and to CR were 7.1 months and 12.2 months, respectively. The median number of RTX cycles required to achieve CR in 85.3% of patients was two. The larynx was the lesional site that took the longest time to achieve disease control. One year after RTX weaning, CR off RTX was obtained in 68.7% of cases. CR off RTX with only minimum doses of immunomodulatory drugs was achieved in 22.0% of patients. Further, 10.1% of patients were partial responders and 4.6% were non-responders to RTX. Relapse occurred in 38.7% of cases, of whom 91.7% had achieved CR again at the last follow-up. In MMP, CR was achieved in a longer time and after more rituximab cycles than in pemphigus, especially for patients with MMP with anti-type VII collagen reactivity. RTX with concomitant immunomodulatory drugs was not responsible for an unusual proportion of adverse events. This large study confirms that RTX is an effective therapy in patients with severe and/or refractory MMP, corroborating previous findings regarding the effects of RTX on AIBDs such as pemphigus.
Subject
Immunology,Immunology and Allergy
Reference48 articles.
1. The First International Consensus on Mucous Membrane Pemphigoid: Definition, Diagnostic Criteria, Pathogenic Factors, Medical Treatment, and Prognostic Indicators;Chan;Arch Dermatol,2002
2. European Guidelines (S3) on Diagnosis and Management of Mucous Membrane Pemphigoid, Initiated by the European Academy of Dermatology and Venereology – Part Ii;Schmidt;J Eur Acad Dermatol Venereol,2021
3. European Guidelines (S3) on Diagnosis and Management of Mucous Membrane Pemphigoid, Initiated by the European Academy of Dermatology and Venereology - Part I;Rashid;J Eur Acad Dermatol Venereol,2021
4. Treatment of Severe Erosive Gingival Lesions by Topical Application of Clobetasol Propionate in Custom Trays;Gonzalez-Moles;Oral Surg Oral Med Oral Pathol Oral Radiol Endod,2003
5. Systemic Minocycline as a Therapeutic Option in Predominantly Oral Mucous Membrane Pemphigoid: A Cautionary Report;Carrozzo;Int J Oral Maxillofac Surg,2009
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