Large International Validation of ABSIS and PDAI Pemphigus Severity Scores

Vivien Hebert 1 Claire Boulard 1 Estelle Houivet 2 Sophie Duvert Lehembre Luca Borradori 3 Rocco Della Torre Claudio Feliciani Luca Fania Giovanna Zambruno 4 Diana Camaioni Biago Didona Branka Marinovic Enno Schmidt Nina Schumacher Christian Hünefeld Stefan Schanz Johannes Steffen Kern Silke Hofmann Anne Charlotte Bouyeure Catherine Picard-Dahan 5 Catherine Prost-Squarcioni 6 Frédéric Caux 7 Marina Alexandre 6 Saskia Ingen-Housz-Oro 8 Martine Bagot 6 Emmanuel Tancrede-Bohin Jean David Bouaziz 9 Nathalie Franck 10 Pierre Vabres 11 Bruno Labeille 12 Marie Richard 13 Emmanuel Delaporte 14 Alain Dupuy 15 Michel d'Incan 16 Gaelle Quéreux 17 François Skowro Carle Paul 18 Cristina Bulai Livideanu Marie Beylot-Barry 19 Marie Sylvie Doutre Martine Avenel-Audran 20 Christophe Bédane 21 Philippe Bernard Laurent Machet Hervé Maillard Denis Jullien 22 Sebastien Debarbieux 23 Bruno Sassolas 24 Laurent Misery 25 Claire Abasq 25 Olivier Dereure 26 Philippe Lagoutte Vincent Ferranti Victoria Werth Dedee Murrell Michael Hertl Jacques Benichou 27 Pascal Joly 28 Michel D’incan
17 CRCINA - Département INCIT - Equipe 2 - Clinical and translational research in skin cancer
CRCINA - Centre de recherche de Cancérologie et d'Immunologie / Nantes - Angers
Abstract : The Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity-Score (ABSIS) scores have been proposed to provide an objective measure of pemphigus activity. These scores have been evaluated only on already treated patients mainly with mild to moderate activity. The objective was to assess the interrater reliability of ABSIS and PDAI scores and their correlation with other severity markers in a large international study. Consecutive patients with newly diagnosed pemphigus were enrolled in 31 centers. Severity scores were recorded during a 24-month period by the same two blinded investigators. Serum was collected at each visit for ELISA measurement of anti-desmoglein antibodies. The intraclass correlation coefficient (ICC) and Spearman rank correlation coefficient were calculated. A total of 116 patients with pemphigus vulgaris (n = 84) or pemphigus foliaceus (n = 32) were included. At baseline, the ABSIS and PDAI ICCs were 0.90 (95% confidence interval [CI] = 0.85-0.93), and 0.91(95% CI = 0.87-0.94), respectively. The ICCs for PDAI were higher in moderate and extensive pemphigus (ICC = 0.82, 95% CI = 0.63-0.92 and ICC = 0.80, 95% CI = 0.62-0.90, respectively) than in patients with intermediate (significant) extent (ICC = 0.50, 95% CI = 0.27-0.68). Conversely, the ICCs for ABSIS were lower in patients with moderate extent (ICC = 0.44, 95% CI = 0.004-0.74) than in those with intermediate or extensive forms, (ICC = 0.69, 95% CI = 0.51-0.81 and ICC = 0.75, 95% CI = 0.51-0.88, respectively). During patients' follow-up, the ICCs of both ABSIS and PDAI scores remained higher than 0.70. ABSIS and PDAI skin (r = 0.71 and r = 0.75) but not mucosal (r = 0.32 and r = 0.37) subscores were correlated with the evolution of anti-DSG1 and anti-DSG3 ELISA values, respectively. ABSIS and PDAI scores are robust tools to accurately assess pemphigus activity.
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Vivien Hebert, Claire Boulard, Estelle Houivet, Sophie Duvert Lehembre, Luca Borradori, et al.. Large International Validation of ABSIS and PDAI Pemphigus Severity Scores. Journal of Investigative Dermatology, Nature Publishing Group, 2019, 139 (1), pp.31-37. ⟨10.1016/j.jid.2018.04.042⟩. ⟨hal-01926791⟩

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