Insights into clinical and diagnostic findings as well as treatment responses in patients with mucous membrane pemphigoid: A retrospective cohort study

Rashid, Hanan; Meijer, Joost; Bolling, Maria C.; Diercks, Gilles F.H.; Pas, Hendri H.; Hórvath, Barbara

doi:10.1016/j.jaad.2021.11.061

Cited by 11 publications

(17 citation statements)

References 40 publications

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“…The median age at MMP diagnosis (69.7 years) was in line with average values reported in other studies ( 3 , 9 , 31 ), but was higher than that in the systematic review by Lytvyn et al ( 11 ). At the time of diagnosis, the most common sites involved in our study in decreasing order of frequency were nasopharyngeal, oral, ocular, laryngeal, genital, and anal MM, which was comparable with previous findings ( 1 , 30 , 31 , 33 ), apart from a higher proportion of nasopharyngeal involvement. In comparison with the study by Lytvyn et al, our series had less frequent ocular involvement (59.6% vs. 70.1%) but more frequent laryngo-tracheal (49.5% vs. 23.9%) and buccal involvement (65.1 vs. 39%), in line with previous publications by our multidisciplinary team ( 34 ).…”

Section: Discussionsupporting

confidence: 92%

“…Previous therapies received by aged population of patients with MMP as well as long term follow-up might have thus contributed to the reported adverse event rates. The 5.5% rate of incident cancer in our current series was similar to that reported in other large series ( 30 ). Moreover, a higher cancer prevalence (11.7%) had been reported previously in a French multicenter retrospective cohort of patients with MMP and was found to not differ from the general population within the same age range in France ( 43 ).…”

Section: Discussionsupporting

confidence: 92%

“…Moreover, a higher cancer prevalence (11.7%) had been reported previously in a French multicenter retrospective cohort of patients with MMP and was found to not differ from the general population within the same age range in France ( 43 ). In concordance with this study ( 43 ), a higher incidence of cancer was not found in patients having anti-laminin 332 antibodies in our studies, whereas it was suggested in other cohorts of patients with MMP ( 30 , 31 , 44 ). Non-infectious and infectious adverse events reported in our cohort included those commonly reported with RTX, notably in other AIBDs such as pemphigus ( 42 ).…”

Section: Discussionsupporting

confidence: 91%

“…Our study population had similar epidemiologic characteristics as those in previous studies; there was a predominance of females (11,(29)(30)(31) as well as diseases lasting for several years before RTX therapy since MMP diagnosis had been delayed for several months to years (28,30,32). The median age at MMP diagnosis (69.7 years) was in line with average values reported in other studies (3, 9, 31), but was higher than that in the systematic review by Lytvyn et al (11).…”

Section: Comparison Of the Study Population With Previous Literaturesupporting

confidence: 88%

“…The population study demonstrated similar severity in comparison with other retrospective studies including patients with MMP, considering the proportion of severe disease (95.4%), patients with ≥3 sites (67.0%) at diagnosis, and patients with multisite involvement (64.2%) at baseline ( 9 , 10 , 27 , 30 ). Regarding therapeutics prior to baseline, fewer patients had been treated with systemic corticosteroids in our study (11.0%) in comparison with the pooled population from the systematic review ( 11 ).…”

Section: Discussionsupporting

confidence: 70%

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Rituximab Therapy for Mucous Membrane Pemphigoid: A Retrospective Monocentric Study With Long-Term Follow-Up in 109 Patients

et al. 2022

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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.

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