International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg
Journal
The British journal of dermatology
ISSN: 1365-2133
Titre abrégé: Br J Dermatol
Pays: England
ID NLM: 0004041
Informations de publication
Date de publication:
12 2021
12 2021
Historique:
accepted:
17
06
2021
pubmed:
27
6
2021
medline:
4
3
2022
entrez:
26
6
2021
Statut:
ppublish
Résumé
European guidelines propose a 0·5 mg kg In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg 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. A 0·5 mg kg
Sections du résumé
BACKGROUND
European guidelines propose a 0·5 mg kg
METHODS
In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg
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
Substances chimiques
Adrenal Cortex Hormones
0
Prednisone
VB0R961HZT
Types de publication
Journal Article
Multicenter Study
Observational Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1232-1239Commentaires et corrections
Type : CommentIn
Informations de copyright
© 2021 British Association of Dermatologists.
Références
Bernard P, Vaillant L, Labeille B et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 1995; 131:48-52.
Zillikens D, Wever S, Roth A et al. Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 1995; 131:957-8.
Gudi VS, White MI, Cruickshank N et al. Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland. Br J Dermatol 2005; 153:424-7.
Joly P, Baricault S, Sparsa A et al. Incidence and mortality of bullous pemphigoid in France. J Invest Dermatol 2012; 132:1998-2004.
Langan SM, Smeeth L, Hubbard R et al. Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: population based cohort study. BMJ 2008; 337:a180.
Jung M, Kippes W, Messer G et al. Increased risk of bullous pemphigoid in male and very old patients: a population-based study on incidence. J Am Acad Dermatol 1999; 41:266-8.
della Torre R, Combescure C, Cortés B et al. Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort. Br J Dermatol 2012; 167:1111-7.
Beissert S, Werfel T, Frieling U et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of bullous pemphigoid. Arch Dermatol 2007; 143:1536-42.
Cordel N, Chosidow O, Hellot M-F et al. Neurological disorders in patients with bullous pemphigoid. Dermatology 2007; 215:187-91.
Roujeau JC, Lok C, Bastuji-Garin S et al. High risk of death in elderly patients with extensive bullous pemphigoid. Arch Dermatol 1998; 134:465-9.
Rzany B, Partscht K, Jung M et al. Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol 2002; 138:903-8.
Bastuji-Garin S, Sbidian E. How to validate outcome instruments for pemphigus. J Invest Dermatol 2009; 129:2328-30.
Joly P, Roujeau J-C, Benichou J et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002; 346:321-7.
Terra JB, Potze WJB, Jonkman MF. Whole body application of a potent topical corticosteroid for bullous pemphigoid. J Eur Acad Dermatol Venereol 2014; 28:712-8.
Feliciani C, Joly P, Jonkman MF et al. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. Br J Dermatol 2015; 172:867-77.
Williams HC, Wojnarowska F, Kirtschig G et al. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. The Lancet 2017; 389:1630-8.
Murrell DF, Daniel BS, Joly P et al. Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts. J Am Acad Dermatol 2012; 66:479-85.
Vaillant L, Bernard P, Joly P et al. Evaluation of clinical criteria for diagnosis of bullous pemphigoid. French Bullous Study Group. Arch Dermatol 1998; 134:1075-80.
Joly P, Courville P, Lok C et al. Clinical criteria for the diagnosis of bullous pemphigoid: a reevaluation according to immunoblot analysis of patient sera. Dermatology 2004; 208:16-20.
Courville P, Kupfer I, Gilbert D et al. [Evaluation of histological criteria for bullous pemphigoid. Correlation with antigens recognized by immunoblotting of anti-epidermal autoantibodies]. Ann Pathol 2000; 20:564-9 (in French).
Roujeau JC, Guillaume JC, Morel P et al. Plasma exchange in bullous pemphigoid. Lancet 1984; 2:486-8.
Masmoudi W, Vaillant M, Vassileva S et al. International validation of the Bullous Pemphigoid Disease Area Index severity score and calculation of cut-off values for defining mild, moderate and severe types of bullous pemphigoid. Br J Dermatol 2021; 184:1106-12.
Tedbirt B, Gillibert A, Andrieu E et al. Mixed individual-aggregate data on all-cause mortality in bullous pemphigoid: a meta-analysis. JAMA Dermatol 2021; 157:421-30.
National Institutes of Health. Common Terminology Criteria for Adverse Events (CTCAE). Available at: https://ctep.cancer.gov/protocoldevelopment/electronic_applications/docs/ctcae_v5_quick_reference_8.5x11.pdf (last accessed 14 July 2021).
Joly P, Roujeau J-C, Benichou J et al. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Invest Dermatol 2009; 129:1681-7.
Joly P, Benichou J, Lok C et al. Prediction of survival for patients with bullous pemphigoid: a prospective study. Arch Dermatol 2005; 141:691-8.