OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. METHODS: We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. RESULTS: More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. CONCLUSIONS: An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process.

4th Department of Paediatrics Aristotle University of Thessaloniki Thessaloniki Greece

Autoinflammatory Alliance San Fransisco USA

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

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

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

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

Department of Paediatric Immunology University Medical Centre Utrecht Utrecht The Netherlands

Department of Paediatric Rheumatic diseases 5 A Nasonova Research Institute of Rheumatology Moscow Russia

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

Department of Paediatric Rheumatology Gulhane Military Medical Faculty Ankara Turkey

Department of Paediatric Rheumatology Hacettepe University Ankara Turkey

Department of Paediatric Rheumatology University Hospital of Geneva Geneva Switzerland

Department of Paediatric Rheumatology University of Lausanne Lausanne Switzerland

Department of Paediatrics Aarhus University Hospital Aarhus Denmark

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

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

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

Department of Paediatrics King Faisal Specialist Hospital and Research Centre Riyadh Saudi Arabia

Department of Paediatrics Meir Medical Centre Kfar Saba Tel Aviv University Sackler School of Medicine Tel Aviv Israel

Department of Paediatrics Rheumatology AOU G Martino Messina Italy

Department of Paediatrics University Medical Centre Utrecht Utrecht The Netherlands

Department of Paediatrics University of California San Diego USA

Department of Rheumatology and Immunology Dr von Hauner Childrens Hospital Ludwig Maximilians University Munich Germany

Dipartimento di Medicina Pediatrica IRCCS Ospedale Pediatrico Bambino Gesù Rome Italy

Direzione Scientifica G Gaslini Institute Genova Italy

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

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

Division of Medicine University College London London UK

Division of Paediatric Rheumatology Department of Paediatrics University Hospital Tuebingen Tuebingen Germany

Division of Rheumatology Ospedale Pediatrico Bambino Gesù Rome Italy

Inflammatory Disease Section National Human Genome Research Institute National Institutes of Health Bethesda 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

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

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 first Paediatric clinic Aristotle University of Thessaloniki Thessaloniki Greece

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

Paediatric Rheumatology unit 4272 Rigshospitalet Copenhagen Denmark

Paediatric Rheumatology Unit Hospital Sant Joan de Déu Barcelona Spain

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

Reference centre for autoinflammatory diseases Versailles Hospital Le Chesnay France

Servicio de Inmunología y Reumatología Hospital de Pediatría Garrahan Buenos Aires Argentina

Translational Autoinflammatory Disease Section NIAID National Institutes of Health Bethesda USA

UOC Pediatria 2 G Gaslini Institute Genova Italy

UOSD Farmacologia Clinica e Clinical Trial Scientific Direction G Gaslini Institute Genova Italy

Ann Rheum Dis. 2017 Aug;76(8):e25. doi: 10.1136/annrheumdis-2016-210901. PubMed

Ann Rheum Dis. 2017 Aug;76(8):e26. doi: 10.1136/annrheumdis-2016-210918. PubMed

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