Large International Validation of ABSIS and PDAI Pemphigus Severity Scores

Vivien Hebert (1) , Claire Boulard (1) , Estelle Houivet (2, 3) , Sophie Duvert Lehembre , Luca Borradori (4) , Rocco Della Torre , Claudio Feliciani , Luca Fania , Giovanna Zambruno (5) , Diana Camaioni , Biago Didona , Branka Marinovic , Enno Schmidt , Nina Schumacher , Christian Hünefeld , Stefan Schanz , Johannes Steffen Kern , Silke Hofmann , Anne Charlotte Bouyeure (3) , Catherine Picard-Dahan (6) , Catherine Prost-Squarcioni (7) , Frédéric Caux (8) , Marina Alexandre (7) , Saskia Ingen-Housz-Oro (9) , Martine Bagot (7) , Emmanuel Tancrede-Bohin , Jean David Bouaziz (10) , Nathalie Franck , Pierre Vabres (11) , Bruno Labeille (12) , Marie Richard (13) , Emmanuel Delaporte (14) , Alain Dupuy (15) , Michel d'Incan (16) , Gaelle Quéreux , François Skowro , Carle F Paul (17) , Cristina Bulai Livideanu , Marie Beylot-Barry (18) , Marie Sylvie Doutre , Martine Avenel-Audran (19) , Christophe Bédane (20) , Philippe Bernard , Laurent Machet , Hervé Maillard , Denis Jullien (21) , Sebastien Debarbieux (22) , Bruno Sassolas (23) , Laurent Misery (24) , Claire Abasq (24) , Olivier Dereure (25, 26) , Philippe Lagoutte (3) , Vincent Ferranti , Victoria Werth , Dedee Murrell , Michael Hertl , Jacques Benichou (27, 3) , Pascal Joly (28) , Michel D’incan

1 UNIROUEN UFR Santé - UNIROUEN - UFR Santé
2 Pharmacoepidemiologie et évaluation de l'impact des produits de santé sur les populations
3 BPH - Bordeaux population health
4 UNIBE - Universität Bern / University of Bern
5 Molecular and Cell Biology Laboratory
6 Service de Dermatologie, Hôpital Bichat, AP-HP
7 Service de dermatologie [Paris]
8 Service de dermatologie [Avicenne]
9 CHU Henri Mondor [Créteil]
10 Service de Dermatologie [AP-HP Hôpital Saint Louis, Paris]
11 Service de Dermatologie (CHU de Dijon)
12 Service de Dermatologie [CHU Saint-Etienne]
13 UGA UFRP - Université Grenoble Alpes - UFR Pharmacie
14 Service de dermatologie (CHRU de Lille)
15 Service de Dermatologie [Rennes] = Dermatology [Rennes]
16 IMoST - Imagerie Moléculaire et Stratégies Théranostiques
17 UT3 - Université Toulouse III - Paul Sabatier
18 Service de dermatologie [Bordeaux]
19 Département de Dermatologie
20 Service de Dermatologie [CHU Limoges]
21 Club Rhumatismes et Inflammation
22 Service de Dermatologie, Centre Hospitalier Sud, Hospices Civils, Lyon
23 DMIP - Brest - Département de Médecine Interne et Pneumologie [Brest]
24 Dermato - BREST - Service de dermatologie
25 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
26 Pathogénèse et contrôle des infections chroniques (PCCI)
27 Génétique médicale et fonctionnelle du cancer et des maladies neuropsychiatriques
28 Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes

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.

Domaines: Cancer

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, 2019, 139 (1), pp.31-37. ⟨10.1016/j.jid.2018.04.042⟩. ⟨hal-01926791⟩