TY - JOUR
T1 - Development of the autoinflammatory disease damage index (ADDI)
AU - ter Haar, Nienke M.
AU - Annink, K.
AU - Al-Mayouf, Sulaiman
AU - Amaryan, Gayane
AU - Anton, Jordi
AU - Barron, Karyl S.
AU - Benseler, Susanne M.
AU - Brogan, Paul
AU - Cantarini, L.
AU - Cattalini, Marco
AU - Cochino, A. V.
AU - De Benedetti, Fabrizio
AU - Dedeoglu, Fatma
AU - De Jesus, Adriana A.
AU - Alberighi, Ornella Della Casa
AU - Demirkaya, Erkan
AU - Dolezalova, Pavla
AU - Durrant, K.
AU - Fabio, Giovanna
AU - Gallizzi, Romina
AU - Goldbach-Mansky, R.
AU - Hachulla, E.
AU - Hentgen, Veronique
AU - Herlin, Troels
AU - Hofer, M.
AU - Hoffman, H.
AU - Insalaco, Antonella
AU - Jansson, Annette
AU - Kallinich, Tilmann
AU - Koné-Paut, Isabelle
AU - Kozlova, Anna
AU - Kuemmerle-Deschner, Jasmin
AU - Lachmann, H.
AU - Laxer, Ronald M.
AU - Martini, Alberto
AU - Nielsen, Susan
AU - Nikishina, Irina
AU - Ombrello, Amanda
AU - Ozen, Seza
AU - Papadopoulou-Alataki, Efimia
AU - Quartier, Pierre
AU - Rigante, D.
AU - Russo, Ricardo
AU - Simon, A.
AU - Trachana, Maria
AU - Uziel, Yosef
AU - Ravelli, Angelo
AU - Gattorno, Marco
AU - Frenkel, J.
PY - 2016/11/3
Y1 - 2016/11/3
N2 - 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 receptorassociated 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.
AB - 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 receptorassociated 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.
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U2 - 10.1136/annrheumdis-2016-210092
DO - 10.1136/annrheumdis-2016-210092
M3 - Article
JO - Annals of the Rheumatic Diseases
JF - Annals of the Rheumatic Diseases
SN - 0003-4967
ER -