International multicentre observational study to assess the efficacy and safety of a 0.5 mg/kg/day starting dose of oral corticosteroids to treat bullous pemphigoid.

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European guidelines propose a 0.5 mg/kg/day dose of oral prednisone as initial treatment of bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent, and patients’general condition.In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0.5 mg/kg/day dose of prednisone, then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0.1 mg/kg/day) within 6 months after the start of treatment. The two co-primary endpoints were control of disease activity at Day21 and one-year overall survival. Disease severity was assessed according to Bullous-Pemphigoid-Disease-Area-Index (BPDAI) score.Between 2015 and 2017, 198 patients were included. The final analysis comprised 190 patients with a mean age of 80.9±9.1 years. Control of disease activity was achieved at Day21 in 119 patients (62.6%; 95%CI=55.3-69.5%): 18 of 24 (75.0%; 95%CI=53.3-90.2%), 75 of 110 (68.2%; 95%CI=59.2-77.3%) and 26 of 56 (46.4%; 95%CI=33.0-60.3%) patients had mild, moderate and severe BP, respectively (p=0.0218). Thirty-two patients died. The overall Kaplan-Meier one-year survival was 82.6 % (95%CI=76.3-87.4%) corresponding to 90.9%, 83.0% and 80.0% rates in patients with mild, moderate and severe BP, respectively (p=0.5). Thresholds of 49-points for BPDAI score and 70-points for Karnofsky score yielded maximal Youden index values with respect to disease control at Day21 and one-year survival, respectively.A 0.5 mg/kg/day dose of prednisone is a valuable therapeutic option in patients with mild/moderate BP whose general condition allows them to be autonomous.

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Authors: V Hébert, S Bastos, K Drenovska, J Meijer, S Ingen-Housz-Oro, C Bedane, L Lunardon, S Debarbieux, H Jedlickova, F Cauxv, G Chaby, M D’Incan, C Feliciani, C Boulard, N Schumacher, E Schmidt, A Roussel, M A Richard, J Gottlieb, V Ferranti, O Guérin, J Bénichou, P Joly, French study Group on auto immune bullous skin diseases, the auto immune bullous skin disease task force of the EADV