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In silico validation of the autoinflammatory disease damage index
Annals of the Rheumatic Diseases
  • Nienke M. Ter Haar, University Medical Center Utrecht
  • Amber Laetitia Justine Van Delft, Wilhelmina Kinderziekenhuis
  • Kim Valerie Annink, Wilhelmina Kinderziekenhuis
  • Henk Van Stel, Wilhelmina Kinderziekenhuis
  • Sulaiman M. Al-Mayouf, King Faisal Specialist Hospital and Research Centre
  • Gayane Amaryan, National Paediatric Centre for Familial Mediterranean Fever and Gastroenterology Service
  • Jordi Anton, Hospital Sant Joan de Déu Barcelona
  • Karyl S. Barron, National Institute of Allergy and Infectious Diseases (NIAID)
  • Susanne Benseler, Alberta Children's Hospital
  • Paul A. Brogan, UCL Great Ormond Street Institute of Child Health
  • Luca Cantarini, Università degli Studi di Siena
  • Marco Cattalini, Spedali Civili Di Brescia
  • Alexis Virgil Cochino, Alessandrescu-Rusescu National Institute of Mother and Child Health
  • Fabrizio De Benedetti, IRCCS Ospedale Pediatrico Bambino Gesù
  • Fatma Dedeoglu, Children's Hospital Boston
  • Adriana Almeida De Jesus, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
  • Erkan Demirkaya, Western University
  • Pavla Dolezalova, Všeobecná Fakultní Nemocnice v Praze
  • Karen L. Durrant, Autoinflammatory Alliance
  • Giovanna Fabio, Ospedale Maggiore Policlinico Milano
  • Romina Gallizzi
  • Raphaela Goldbach-Mansky, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
  • Eric Hachulla, Université de Lille
  • Veronique Hentgen, CHV Centre Hospitalier de Versailles
  • Troels Herlin, Aarhus Universitetshospital
  • Michaël Hofer, Université de Lausanne (UNIL)
  • Hal M. Hoffman, University of California, San Diego
  • Antonella Insalaco, IRCCS Ospedale Pediatrico Bambino Gesù
  • Annette F. Jansson, Charité – Universitätsmedizin Berlin
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Introduction Autoinflammatory diseases can cause irreversible tissue damage due to systemic inflammation. Recently, the Autoinflammatory Disease Damage Index (ADDI) was developed. The ADDI is the first instrument to quantify damage in familial Mediterranean fever, cryopyrin-associated periodic syndromes, mevalonate kinase deficiency and tumour necrosis factor receptor-associated periodic syndrome. The aim of this study was to validate this tool for its intended use in a clinical/research setting. Methods The ADDI was scored on paper clinical cases by at least three physicians per case, independently of each other. Face and content validity were assessed by requesting comments on the ADDI. Reliability was tested by calculating the intraclass correlation coefficient (ICC) using an â observer-nested-within-subject' design. Construct validity was determined by correlating the ADDI score to the Physician Global Assessment (PGA) of damage and disease activity. Redundancy of individual items was determined with Cronbach's alpha. Results The ADDI was validated on a total of 110 paper clinical cases by 37 experts in autoinflammatory diseases. This yielded an ICC of 0.84 (95% CI 0.78 to 0.89). The ADDI score correlated strongly with PGA-damage (r=0.92, 95% CI 0.88 to 0.95) and was not strongly influenced by disease activity (r=0.395, 95% CI 0.21 to 0.55). After comments from disease experts, some item definitions were refined. The interitem correlation in all different categories was lower than 0.7, indicating that there was no redundancy between individual damage items. Conclusion The ADDI is a reliable and valid instrument to quantify damage in individual patients and can be used to compare disease outcomes in clinical studies.

Citation Information
Nienke M. Ter Haar, Amber Laetitia Justine Van Delft, Kim Valerie Annink, Henk Van Stel, et al.. "In silico validation of the autoinflammatory disease damage index" Annals of the Rheumatic Diseases Vol. 77 Iss. 11 (2018) p. 1599 - 1605
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