RT Journal Article
SR Electronic
T1 Development of the autoinflammatory disease damage index (ADDI)
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 821
OP 830
DO 10.1136/annrheumdis-2016-210092
VO 76
IS 5
A1 Nienke M ter Haar
A1 Kim V Annink
A1 Sulaiman M Al-Mayouf
A1 Gayane Amaryan
A1 Jordi Anton
A1 Karyl S Barron
A1 Susanne M Benseler
A1 Paul A Brogan
A1 Luca Cantarini
A1 Marco Cattalini
A1 Alexis-Virgil Cochino
A1 Fabrizio De Benedetti
A1 Fatma Dedeoglu
A1 Adriana A De Jesus
A1 Ornella Della Casa Alberighi
A1 Erkan Demirkaya
A1 Pavla Dolezalova
A1 Karen L Durrant
A1 Giovanna Fabio
A1 Romina Gallizzi
A1 Raphaela Goldbach-Mansky
A1 Eric Hachulla
A1 Veronique Hentgen
A1 Troels Herlin
A1 Michaël Hofer
A1 Hal M Hoffman
A1 Antonella Insalaco
A1 Annette F Jansson
A1 Tilmann Kallinich
A1 Isabelle Koné-Paut
A1 Anna Kozlova
A1 Jasmin B Kuemmerle-Deschner
A1 Helen J Lachmann
A1 Ronald M Laxer
A1 Alberto Martini
A1 Susan Nielsen
A1 Irina Nikishina
A1 Amanda K Ombrello
A1 Seza Ozen
A1 Efimia Papadopoulou-Alataki
A1 Pierre Quartier
A1 Donato Rigante
A1 Ricardo Russo
A1 Anna Simon
A1 Maria Trachana
A1 Yosef Uziel
A1 Angelo Ravelli
A1 Marco Gattorno
A1 Joost Frenkel
YR 2017
UL http://ard.bmj.com/content/76/5/821.abstract
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 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.