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Large International Validation of ABSIS and PDAI Pemphigus Severity Scores.

  • Hébert V Department of Dermatology, Rouen University Hospital, and INSERM U 1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France. Electronic address: Vivien.hebert@chu-rouen.fr.
  • Boulard C Department of Dermatology, Rouen University Hospital, and INSERM U 1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France.
  • Houivet E Department of Biostatistics and Clinical Research, INSERM U 1219, Rouen University Hospital, University of Rouen, Rouen, France.
  • Duvert Lehembre S Department of Dermatology, Lille University Hospital, Lille, France.
  • Borradori L Department of Dermatology, Universitätsklinik für Dermatologie Inselspital, Bern, Switzerland.
  • Della Torre R Department of Dermatology, Universitätsklinik für Dermatologie Inselspital, Bern, Switzerland.
  • Feliciani C Dermatology Unit, Università di Parma, Parma, Italy.
  • Fania L Policlinico Gemelli-Università Cattolica del "Sacro Cuore," Rome, Italy.
  • Zambruno G Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico, Rome, Italy.
  • Camaioni DB Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico, Rome, Italy.
  • Didona B Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico, Rome, Italy.
  • Marinovic B Department of Dermatology and Venereology, University Hospital Center Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia.
  • Schmidt E Comprehensive Center for Inflammation Medicine, Department of Dermatology, University of Luebeck Ratzeburger, Luebeck, Germany.
  • Schumacher N Comprehensive Center for Inflammation Medicine, Department of Dermatology, University of Luebeck Ratzeburger, Luebeck, Germany.
  • Hünefeld C Universitäts-Hautklinik Tübingen, Tübingen, Germany.
  • Schanz S Universitäts-Hautklinik Tübingen, Tübingen, Germany.
  • Kern JS Department of Dermatology, Medical Center, University of Freiburg, Freiburg, Germany.
  • Hofmann S Department of Dermatology, Medical Center, University of Freiburg, Freiburg, Germany.
  • Bouyeure AC Department of Biostatistics and Clinical Research, INSERM U 1219, Rouen University Hospital, University of Rouen, Rouen, France.
  • Picard-Dahan C Department of Dermatology, Bichat University Hospital, (Assistance Publique - Hôpitaux de Paris), Paris, France.
  • Prost-Squarcioni C Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France.
  • Caux F Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France.
  • Alexandre M Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France.
  • Ingen-Housz-Oro S Department of Dermatology, Henri Mondor University Hospital, (Assistance Publique - Hôpitaux de Paris), Créteil, France.
  • Bagot M Department of Dermatology, Saint Louis Hospital, (Assistance Publique - Hôpitaux de Paris), Paris, France.
  • Tancrede-Bohin E Department of Dermatology, Saint Louis Hospital, (Assistance Publique - Hôpitaux de Paris), Paris, France.
  • Bouaziz JD Department of Dermatology, Saint Louis Hospital, (Assistance Publique - Hôpitaux de Paris), Paris, France.
  • Franck N Department of Dermatology, Cochin Hospital, (Assistance Publique - Hôpitaux de Paris), Paris, France.
  • Vabres P Department of Dermatology, Dijon University Hospital, Dijon, France.
  • Labeille B Department of Dermatology, Saint Etienne University Hospital, Saint Etienne, France.
  • Richard MA Aix-Marseille University, UMR 911, INSERM CRO2, "Centre de recherche en oncologie biologique et oncophamacologie," Department of Dermatology, Hôpital Timone, Assistance Publique des Hôpitaux de Marseille, Marseille, France.
  • Delaporte E Department of Dermatology, Lille University Hospital, Lille, France.
  • Dupuy A Department of Dermatology, Rennes University Hospital, Rennes, France.
  • D'Incan M Department of Dermatology, Clermond-Ferrand University Hospital, Clermont-Ferrand, France.
  • Quereux G Department of Dermatology, Nantes University Hospital, Nantes, France.
  • Skowro F Department of Dermatology, Valence Hospital, Valence, France.
  • Paul C Department of Dermatology, Toulouse University and INSERM U1056, Toulouse, France.
  • Livideanu CB Department of Dermatology, Toulouse University and INSERM U1056, Toulouse, France.
  • Beylot-Barry M Department of Dermatology, Bordeaux University Hospital, Bordeaux, France.
  • Doutre MS Department of Dermatology, Bordeaux University Hospital, Bordeaux, France.
  • Avenel-Audran M Department of Dermatology, Angers University Hospital, Angers, France.
  • Bedane C Department of Dermatology, Limoges University Hospital, Limoges, France.
  • Bernard P Department of Dermatology, Reims University Hospital, Reims, France.
  • Machet L Department of Dermatology, Tours University Hospital, Tours, France.
  • Maillard H Department of Dermatology, Le Mans Hospital, Le Mans, France.
  • Jullien D Department of Dermatology, Lyon University Hospital, Lyon, France.
  • Debarbieux S Department of Dermatology, Lyon University Hospital, Lyon, France.
  • Sassolas B Department of Dermatology, Brest University Hospital, Brest, France.
  • Misery L Department of Dermatology, Brest University Hospital, Brest, France.
  • Abasq C Department of Dermatology, Brest University Hospital, Brest, France.
  • Dereure O Department of Dermatology, Montpellier University Hospital, Montpellier, France.
  • Lagoutte P Department of Biostatistics and Clinical Research, INSERM U 1219, Rouen University Hospital, University of Rouen, Rouen, France.
  • Ferranti V Department of Dermatology, Rouen University Hospital, and INSERM U 1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France.
  • Werth VP Department of Dermatology, University of Pennsylvania, and Philadelphia Veterans Affairs Medical Center, Philadelphia, Pennsylvania, USA.
  • Murrell DF Department of Dermatology, St George Hospital, University of NSW, Sydney, Australia.
  • Hertl M Department of Dermatology, Philipps University Marburg, Marburg, Germany.
  • Benichou J Department of Biostatistics and Clinical Research, INSERM U 1219, Rouen University Hospital, University of Rouen, Rouen, France.
  • Joly P Department of Dermatology, Rouen University Hospital, and INSERM U 1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France.
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  • 2018-10-11
Published in:
  • The Journal of investigative dermatology. - 2019
Autoantibodies
Autoimmunity
Desmoglein 1
Humans
Pemphigus
Severity of Illness Index
Skin
Validation Studies as Topic
English 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.
Language
  • English
Open access status
bronze
Identifiers
Persistent URL
https://sonar.ch/global/documents/278812
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