Development of the autoinflammatory diseases damage index (ADDI)

Donato Rigante, Nienke M. Ter Haar, Kim V. Annink, Sulaiman M. Al-Mayouf, Gayane Amaryan, Jordi Anton, Karyl S. Barron, Susanne M. Benseler, Paul A. Brogan, Luca Cantarini, Marco Cattalini, Alexis-Virgil Cochino, Fabrizio De Benedetti, Fatma Dedeoglu, Adriana A. De Jesus, Ornella Della Casa Alberighi, Erkan Demirkaya, Pavla Dolezalova, Karen L. Durrant, Giovanna FabioRomina Gallizzi, Raphaela Goldbach-Mansky, Eric Hachulla, Veronique Hentgen, Troels Herlin, Michaël Hofer, Hal M. Hoffman, Antonella Insalaco, Annette F. Jansson, Tilmann Kallinich, Isabelle Koné-Paut, Anna Kozlova, Jasmin B. Kuemmerle-Deschner, Helen J. Lachmann, Ronald M. Laxer, Alberto Martini, Susan Nielsen, Irina Nikishina, Amanda K. Ombrello, Seza Ozen, Efimia Papadopoulou-Alataki, Pierre Quartier, Ricardo Russo, Anna Simon, Maria Trachana, Yosef Uziel, Angelo Ravelli, Marco Gattorno, Joost Frenkel

Risultato della ricerca: Contributo in rivistaArticolo in rivista

42 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)1-10
Numero di pagine10
RivistaANNALS OF THE RHEUMATIC DISEASES
Volume2016
DOI
Stato di pubblicazionePubblicato - 2016

Keywords

  • Autoinflammation

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