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Ann Rheum Dis. 2017 May;76(5):821-830. doi: 10.1136/annrheumdis-2016-210092. Epub 2016 Nov 3.

Development of the autoinflammatory disease damage index (ADDI).

Author information

1
Laboratory for Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands.
2
Department of Paediatric Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands.
3
Department of Paediatrics, University Medical Centre Utrecht, Utrecht, The Netherlands.
4
Department of Paediatrics, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
5
National Paediatric Centre for Familial Mediterranean Fever and Gastroenterology Service, Arabkir Medical Centre-Institute of Child & Adolescent Health, Yerevan, Armenia.
6
Paediatric Rheumatology Unit, Hospital Sant Joan de Déu, Barcelona, Spain.
7
Division of Intramural Research and National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, USA.
8
Department of Paediatrics and Department of Rheumatology, Alberta Children's Hospital, Calgary, Canada.
9
Department of Infection, Inflammation and Rheumatology, University College London Institute of Child Health, London, UK.
10
Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena, Siena, Italy.
11
Paediatric Clinic, University of Brescia and Spedali Civili di Brescia, Brescia, Italy.
12
Paediatrics Department, National Institute for Mother and Child Health Alessandrescu-Rusescu, Bucharest, Romania.
13
Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
14
Division of Immunology, Rheumatology Program, Boston Children's Hospital, Harvard Medical School, Boston, USA.
15
Translational Autoinflammatory Disease Section, NIAID, National Institutes of Health, Bethesda, USA.
16
UOSD Farmacologia Clinica e Clinical Trial-Scientific Direction, G. Gaslini Institute, Genova, Italy.
17
Department of Paediatric Rheumatology, Gulhane Military Medical Faculty, Ankara, Turkey.
18
Department of Paediatrics and Adolescent Medicine, Charles University, General University Hospital, Prague, Czech Republic.
19
Autoinflammatory Alliance, San Fransisco, USA.
20
Department of Internal Medicine, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
21
Department of Paediatrics, Rheumatology, AOU G Martino, Messina, Italy.
22
Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France.
23
Reference centre for autoinflammatory diseases (CEREMAI), Versailles Hospital, Le Chesnay, France.
24
Department of Paediatrics, Aarhus University Hospital, Aarhus, Denmark.
25
Department of Paediatric Rheumatology, University of Lausanne, Lausanne, Switzerland.
26
Department of Paediatric Rheumatology, University Hospital of Geneva, Geneva, Switzerland.
27
Department of Paediatrics, University of California, San Diego, USA.
28
Dipartimento di Medicina Pediatrica, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
29
Department of Rheumatology&Immunology, Dr. von Hauner Childrens Hospital, Ludwig-Maximilians-University, Munich, Germany.
30
Paediatric Pneumology and Immunology and Interdisciplinary Centre for Social Paediatrics, Charité University Medicine Berlin, Berlin, Germany.
31
Department of Paediatric Rheumatology and CEREMAI, Bicêtre Hospital, APHP, University of Paris Sud, Paris, France.
32
Department of Immunology, Federal Research and Clinical Centre for Paediatric Haematology, Oncology and Immunology, Moscow, Russia.
33
Division of Paediatric Rheumatology, Department of Paediatrics, University Hospital Tuebingen, Tuebingen, Germany.
34
Division of Medicine, University College London, London, UK.
35
Department of Paediatrics and Medicine, University of Toronto and the Hospital for Sick Children, Toronto, Canada.
36
Direzione Scientifica, G Gaslini Institute, Genova, Italy.
37
Paediatric Rheumatology unit 4272, Rigshospitalet, Copenhagen, Denmark.
38
Department of Paediatric Rheumatic diseases, V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
39
Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, USA.
40
Department of Paediatric Rheumatology, Hacettepe University, Ankara, Turkey.
41
Fourth Department of Paediatrics, Aristotle University of Thessaloniki, Thessaloniki, Greece.
42
Department of Paediatric Immunology-Hematology and Rheumatology Unit and IMAGINE Institute, Institution Necker-Enfants Malades Hospital and Paris-Descartes University, Paris, France.
43
Institute of Paediatrics, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy.
44
Servicio de Inmunología y Reumatología, Hospital de Pediatría Garrahan, Buenos Aires, Argentina.
45
Internal Medicine, Radboud Expertise Centre for Immunodeficiency and Autoinflammation, Radboud University Medical Centre, Nijmegen, The Netherlands.
46
Paediatric Immunology and Rheumatology Referral Centre, first Paediatric clinic, Aristotle University of Thessaloniki, Thessaloniki, Greece.
47
Department of Paediatrics, Meir Medical Centre, Kfar Saba, Tel Aviv University, Sackler School of Medicine, Tel Aviv, Israel.
48
Institution Università degli Studi di Genova and G. Gaslini Institute, Genova, Italy.
49
UOC Pediatria 2, G. Gaslini Institute, Genova, Italy.

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.

KEYWORDS:

Familial Mediterranean Fever; Fever Syndromes; Outcomes research; Patient perspective

PMID:
27811147
DOI:
10.1136/annrheumdis-2016-210092
[Indexed for MEDLINE]
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