title: Validation of the auto-inflammatory diseases activity index (AIDAI) for hereditary recurrent fever syndromes.
abstract: OBJECTIVE
  To validate the Auto-Inflammatory Diseases Activity Index (AIDAI) in the four major hereditary recurrent fever syndromes (HRFs): familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), tumour necrosis factor receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndromes (CAPS).
  
  
  METHODS
  In 2010, an international collaboration established the content of a disease activity tool for HRFs. Patients completed a 1-month prospective diary with 12 yes/no items before a clinical appointment during which their physician assessed their disease activity by a questionnaire. Eight international experts in auto-inflammatory diseases evaluated the patient's disease activity by a blinded web evaluation and a nominal group technique consensus conference, with their consensus judgement considered the gold standard. Sensitivity/specificity/accuracy measures and the ability of the score to discriminate active from inactive patients via the best cut-off score were calculated by a receiver operating characteristic analysis.
  
  
  RESULTS
  Consensus was achieved for 98/106 (92%) cases (39 FMF, 35 CAPS, 14 TRAPS and 10 MKD), with 26 patients declared as having inactive disease and 72 as having active disease. The median total AIDAI score was 14 (range=0-175). An AIDAI cut-off score ≥9 discriminated active from inactive patients, with sensitivity/specificity/accuracy of 89%/92%/90%, respectively, and an area under the curve of 98% (95% CI 96% to 100%).
  
  
  CONCLUSIONS
  The AIDAI score is a valid and simple tool for assessing disease activity in FMF/MKD/TRAPS/CAPS. This tool is easy to use in clinical practice and has the potential to be used as the standard efficacy measure in future clinical trials.
authors.affiliation: Service de Pédiatrie Générale et Rhumatologie pédiatrique, Centre de référence des maladies auto-inflammatoires de l'enfant (CeRéMAI), CHU de Bicêtre, APHP, Université Paris Sud, Le Kremlin Bicêtre, France.
authors.forename: Maryam
authors.initials: M
authors.lastname: Piram
authors.affiliation: Service de Pédiatrie Générale et Rhumatologie pédiatrique, Centre de référence des maladies auto-inflammatoires de l'enfant (CeRéMAI), CHU de Bicêtre, APHP, Université Paris Sud, Le Kremlin Bicêtre, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Koné-Paut
authors.affiliation: National Amyloidosis Centre, Royal Free Campus, University College London Medical School, London, UK.
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.affiliation: Department of Paediatrics, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Joost
authors.initials: J
authors.lastname: Frenkel
authors.affiliation: Pediatric Rheumatology, Hacettepe University Children's Hospital, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Division of Pediatric Rheumatology, Universitätsklinik für Kinderheilkunde und Jugendmedizin, Tübingen, Germany.
authors.forename: Jasmin
authors.initials: J
authors.lastname: Kuemmerle-Deschner
authors.affiliation: Department of Infectious Diseases and Immunology, Children's Hospital, University of Munich, Munich, Germany.
authors.forename: Silvia
authors.initials: S
authors.lastname: Stojanov
authors.affiliation: Department of General Internal Medicine, N4i Centre for Immunodeficiency and Autoinflammation (NCIA), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Anna
authors.initials: A
authors.lastname: Simon
authors.affiliation: Pediatria II, Reumatologia, Istituto G Gaslini, PRINTO Coordinating Centre, Genova, Italy.
authors.forename: Martina
authors.initials: M
authors.lastname: Finetti
authors.affiliation: Dipartimento di Scienze della Salute, Università di Genova, Genova, Italy.
authors.forename: Maria Pia
authors.initials: MP
authors.lastname: Sormani
authors.affiliation: Pediatria II, Reumatologia, Istituto G Gaslini, PRINTO Coordinating Centre, Genova, Italy Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.affiliation: Pediatria II, Reumatologia, Istituto G Gaslini, PRINTO Coordinating Centre, Genova, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
authors.affiliation: Pediatria II, Reumatologia, Istituto G Gaslini, PRINTO Coordinating Centre, Genova, Italy.
authors.forename: Nicolino
authors.initials: N
authors.lastname: Ruperto
mesh_terms: D000293:Adolescent; D000328:Adult; D019540:Area Under Curve; D002648:Child; D015331:Cohort Studies; D056587:Cryopyrin-Associated Periodic Syndromes / Q000175:diagnosis / Q000503:physiopathology; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D005334:Fever; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D008297:Male; D054078:Mevalonate Kinase Deficiency / Q000175:diagnosis / Q000503:physiopathology; D011446:Prospective Studies; D012372:ROC Curve; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D055815:Young Adult
keywords: Disease Activity; Familial Mediterranean Fever; Fever Syndromes
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