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 '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.

4th Department of Pediatrics Aristotle University of Thessaloniki Thessaloniki Greece

Autoinflammatory Alliance San Francisco California USA

Département de Médecine Interne et Immunologie Clinique Université de Lille Lille France

Department of General Internal Medicine Radboud Expertise Centre for Immunodeficiency and Autoinflammation Radboud University Medical Centre Nijmegen The Netherlands

Department of Immunology Federal Research and Clinical Centre for Paediatric Haematology Oncology and Immunology Moscow Russian Federation

Department of Infection Inflammation and Rheumatology University College London Institute of Child Health London UK

Department of Internal Medicine Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano Italy

Department of Paediatric Immunology and Rheumatology University Medical Centre Utrecht Utrecht The Netherlands

Department of Paediatric Immunology Hematology and Rheumatology Unit and IMAGINE Institute Institution Necker Enfants Malades Hospital and Paris Descartes University Paris Île de France France

Department of Paediatric Rheumatic Diseases VA Nasonova Research Institute of Rheumatology Moscow Russian Federation

Department of Paediatric Rheumatology and CEREMAI Bicêtre Hospital APHP University of Paris Sud Paris France

Department of Paediatric Rheumatology AOUG Martino Messina Italy

Department of Paediatric Rheumatology University of Lausanne Lausanne Switzerland

Department of Paediatrics and Adolescent Medicine Charles University General University Hospital Praha Czech Republic

Department of Paediatrics and Medicine University of Toronto and the Hospital for Sick Children Toronto Ontario Canada

Department of Paediatrics Meir Medical Centre Kfar Saba Israel

Department of Paediatrics National Institute for Mother and Child Health Alessandrescu Rusescu Bucharest Romania

Department of Paediatrics Universitair Medisch Centrum Utrecht Locatie Wilhelmina Kinderziekenhuis Utrecht The Netherlands

Department of Paediatrics University of California San Diego California USA

Department of Pediatrics King Faisal Specialist Hospital and Research Center Riyadh Saudi Arabia

Department of Pediatrics University of Cincinnati Cincinnati Ohio USA

Department of Rheumatology and Immunology Dr von Hauner Children's Hospital Ludwig Maximilians University Munich Germany

Departmentof Paediatric Rheumatology University Hospital of Geneva Geneva Switzerland

Departmentof Paediatrics Aarhus University Hospital Aarhus Denmark

Departments of Paediatrics and Rheumatology Alberta Children's Hospital Calgary Alberta Canada

Dipartimento di Medicina Pediatrica IRCCS Ospedale Pediatrico Bambino Gesù Roma Italy

Direzione Scientifica Istituto Giannina Gaslini Genova Liguria Italy

Division of Intramural Research and National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda Maryland USA

Division of Medicine University College London London UK

Division of Pediatric Rheumatology Department of Pediatrics University Hospital Tübingen Tübingen Germany

Division of Rheumatology Cincinnati Children's Hospital Medical Center Cincinnati Ohio USA

Division of Rheumatology Ospedale Pediatrico Bambino Gesù Rome Italy

Inflammatory Disease Section National Human Genome Research Institute Bethesda Maryland USA

Institute of Paediatrics Fondazione Policlinico Universitario A Gemelli Università Cattolica Sacro Cuore Rome Italy

Institution Università degli Studi di Genova and G Gaslini Institute Genova Italy

Laboratory for Translational Immunology University Medical Centre Utrecht Utrecht The Netherlands

National Paediatric Centre for Familial Mediterranean Fever and Gastroenterology Service Arabkir Medical Centre Institute of Child and Adolescent Health Yerevan Armenia

Paediatric Clinic University of Brescia and Spedali Civili di Brescia Brescia Italy

Paediatric Immunology and Rheumatology Referral Centre 1st Paediatric Clinic Aristotle University of Thessaloniki Thessaloniki Greece

Paediatric Pneumology and Immunology and Interdisciplinary Centre for Social Paediatrics Charite University Medicine Berlin Berlin Germany

Paediatric Rheumatology Unit 4272 Rigshospitalet Copenhagen Denmark

Paediatric Rheumatology Unit Hospital Sant Joan de Déu Barcelona Spain

Pediatric Rheumatology Hacettepe University Ankara Turkey

Reference Centre for Autoinflammatory Diseases Versailles Hospital Le Chesnay France

Rheumatology Program Division of Immunology Boston Children's Hospital Harvard Medical School Boston Massachusetts USA

Rheumatology Unit Department of Medical Sciences Surgery and Neurosciences University of Siena Siena Italy

Servicio de Inmunología Reumatología Hospital de Pediatria Juan P Garrahan Buenos Aires Argentina

Translational Autoinflammatory Disease Section NIAMS NIH Bethesda Maryland USA

Western University Children's Hospital London Health Sciences Centre London UK

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