International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg−1 per day starting dose of oral corticosteroids to treat bullous pemphigoid

for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology

doi:10.1111/bjd.20593

International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg⁻¹ per day starting dose of oral corticosteroids to treat bullous pemphigoid

for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology

7 Citations (Scopus)

Abstract

Background: European guidelines propose a 0·5 mg kg⁻¹ per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients. Methods: In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg⁻¹ per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0·1 mg kg⁻¹ per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score. Results: In total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3–69.5]; 18 of 24 patients (75%, 95% CI 53·3–90·2), 75 of 110 patients (68·8%, 95% CI 59·2–77·3) and 26 of 56 patients (46.4%, 95% CI 33·0–60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan–Meier 1-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 day 21 and 1-year survival, respectively. Conclusions: A 0·5 mg kg⁻¹ per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous.

Original language	English
Journal	British Journal of Dermatology
Volume	185
Issue number	6
Pages (from-to)	1232-1239
Number of pages	8
ISSN	0007-0963
DOIs	https://doi.org/10.1111/bjd.20593
Publication status	Published - 12.2021

Research Areas and Centers

Academic Focus: Center for Infection and Inflammation Research (ZIEL)
Centers: Center for Research on Inflammation of the Skin (CRIS)

DFG Research Classification Scheme

2.21-05 Immunology
2.22-19 Dermatology
2.22-09 Pharmacology
2.22-22 Clinical Immunology and Allergology

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1111/bjd.20593

Cite this

for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology (2021). International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg⁻¹ per day starting dose of oral corticosteroids to treat bullous pemphigoid. British Journal of Dermatology, 185(6), 1232-1239. https://doi.org/10.1111/bjd.20593

for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology. / International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg⁻¹ per day starting dose of oral corticosteroids to treat bullous pemphigoid. In: British Journal of Dermatology. 2021 ; Vol. 185, No. 6. pp. 1232-1239.

@article{78be6feeba774e5a9666332adccc100f,

title = "International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg−1 per day starting dose of oral corticosteroids to treat bullous pemphigoid",

abstract = "Background: European guidelines propose a 0·5 mg kg−1 per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients. Methods: In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg−1 per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0·1 mg kg−1 per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score. Results: In total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3–69.5]; 18 of 24 patients (75%, 95% CI 53·3–90·2), 75 of 110 patients (68·8%, 95% CI 59·2–77·3) and 26 of 56 patients (46.4%, 95% CI 33·0–60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan–Meier 1-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 day 21 and 1-year survival, respectively. Conclusions: A 0·5 mg kg−1 per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous.",

author = "{for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology} and V. H{\'e}bert and S. Bastos and K. Drenovska and J. Meijer and S. Ingen-Housz-Oro and C. Bedane and L. Lunardon and S. Debarbieux and H. Jedlickova and F. Caux and G. Chaby and M. D{\textquoteright}Incan and C. Feliciani and C. Boulard and N. Schumacher and E. Schmidt and A. Roussel and Richard, {M. A.} and J. Gottlieb and V. Ferranti and O. Gu{\'e}rin and J. B{\'e}nichou and P. Joly",

note = "Publisher Copyright: {\textcopyright} 2021 British Association of Dermatologists",

year = "2021",

month = dec,

doi = "10.1111/bjd.20593",

language = "English",

volume = "185",

pages = "1232--1239",

journal = "British Journal of Dermatology",

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for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology 2021, 'International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg⁻¹ per day starting dose of oral corticosteroids to treat bullous pemphigoid', British Journal of Dermatology, vol. 185, no. 6, pp. 1232-1239. https://doi.org/10.1111/bjd.20593

International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg⁻¹ per day starting dose of oral corticosteroids to treat bullous pemphigoid. / for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology.
In: British Journal of Dermatology, Vol. 185, No. 6, 12.2021, p. 1232-1239.

Research output: Journal Articles › Journal articles › Research › peer-review

TY - JOUR

T1 - International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg−1 per day starting dose of oral corticosteroids to treat bullous pemphigoid

AU - for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology

AU - Hébert, V.

AU - Bastos, S.

AU - Drenovska, K.

AU - Meijer, J.

AU - Ingen-Housz-Oro, S.

AU - Bedane, C.

AU - Lunardon, L.

AU - Debarbieux, S.

AU - Jedlickova, H.

AU - Caux, F.

AU - Chaby, G.

AU - D’Incan, M.

AU - Feliciani, C.

AU - Boulard, C.

AU - Schumacher, N.

AU - Schmidt, E.

AU - Roussel, A.

AU - Richard, M. A.

AU - Gottlieb, J.

AU - Ferranti, V.

AU - Guérin, O.

AU - Bénichou, J.

AU - Joly, P.

PY - 2021/12

Y1 - 2021/12

N2 - Background: European guidelines propose a 0·5 mg kg−1 per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients. Methods: In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg−1 per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0·1 mg kg−1 per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score. Results: In total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3–69.5]; 18 of 24 patients (75%, 95% CI 53·3–90·2), 75 of 110 patients (68·8%, 95% CI 59·2–77·3) and 26 of 56 patients (46.4%, 95% CI 33·0–60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan–Meier 1-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 day 21 and 1-year survival, respectively. Conclusions: A 0·5 mg kg−1 per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous.

AB - Background: European guidelines propose a 0·5 mg kg−1 per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients. Methods: In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg−1 per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0·1 mg kg−1 per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score. Results: In total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3–69.5]; 18 of 24 patients (75%, 95% CI 53·3–90·2), 75 of 110 patients (68·8%, 95% CI 59·2–77·3) and 26 of 56 patients (46.4%, 95% CI 33·0–60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan–Meier 1-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 day 21 and 1-year survival, respectively. Conclusions: A 0·5 mg kg−1 per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous.

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U2 - 10.1111/bjd.20593

DO - 10.1111/bjd.20593

M3 - Journal articles

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SN - 0007-0963

VL - 185

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JO - British Journal of Dermatology

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ER -

for the French study group on autoimmune bullous skin diseases, and the autoimmune bullous skin disease task force of the European Academy of Dermatology and Venereology. International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg⁻¹ per day starting dose of oral corticosteroids to treat bullous pemphigoid. British Journal of Dermatology. 2021 Dec;185(6):1232-1239. doi: 10.1111/bjd.20593