@article {ter Haarannrheumdis-2016-210092, author = {Nienke M ter Haar and Kim V Annink and Sulaiman M Al-Mayouf and Gayane Amaryan and Jordi Anton and Karyl S Barron and Susanne M Benseler and Paul A Brogan and Luca Cantarini and Marco Cattalini and Alexis-Virgil Cochino and Fabrizio De Benedetti and Fatma Dedeoglu and Adriana A De Jesus and Ornella Della Casa Alberighi and Erkan Demirkaya and Pavla Dolezalova and Karen L Durrant and Giovanna Fabio and Romina Gallizzi and Raphaela Goldbach-Mansky and Eric Hachulla and Veronique Hentgen and Troels Herlin and Micha{\"e}l Hofer and Hal M Hoffman and Antonella Insalaco and Annette F Jansson and Tilmann Kallinich and Isabelle Kon{\'e}-Paut and Anna Kozlova and Jasmin B Kuemmerle-Deschner and Helen J Lachmann and Ronald M Laxer and Alberto Martini and Susan Nielsen and Irina Nikishina and Amanda K Ombrello and Seza Ozen and Efimia Papadopoulou-Alataki and Pierre Quartier and Donato Rigante and Ricardo Russo and Anna Simon and Maria Trachana and Yosef Uziel and Angelo Ravelli and Marco Gattorno and Joost Frenkel}, title = {Development of the autoinflammatory disease damage index (ADDI)}, elocation-id = {annrheumdis-2016-210092}, year = {2016}, doi = {10.1136/annrheumdis-2016-210092}, publisher = {BMJ Publishing Group Ltd}, abstract = {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.}, issn = {0003-4967}, URL = {https://ard.bmj.com/content/early/2016/11/03/annrheumdis-2016-210092}, eprint = {https://ard.bmj.com/content/early/2016/11/03/annrheumdis-2016-210092.full.pdf}, journal = {Annals of the Rheumatic Diseases} }