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Extended report
Validation of the Auto-Inflammatory Diseases Activity Index (AIDAI) for hereditary recurrent fever syndromes
  1. Maryam Piram1,
  2. Isabelle Koné-Paut1,
  3. Helen J Lachmann2,
  4. Joost Frenkel3,
  5. Seza Ozen4,
  6. Jasmin Kuemmerle-Deschner5,
  7. Silvia Stojanov6,
  8. Anna Simon7,
  9. Martina Finetti8,
  10. Maria Pia Sormani9,
  11. Alberto Martini8,10,
  12. Marco Gattorno8,
  13. Nicolino Ruperto8
  14. on the behalf of EUROFEVER, EUROTRAPS and the Paediatric Rheumatology International Trials Organisation (PRINTO) networks
  1. 1Service 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
  2. 2National Amyloidosis Centre, Royal Free Campus, University College London Medical School, London, UK
  3. 3Department of Paediatrics, University Medical Center Utrecht, Utrecht, The Netherlands
  4. 4Pediatric Rheumatology, Hacettepe University Children's Hospital, Ankara, Turkey
  5. 5Division of Pediatric Rheumatology, Universitätsklinik für Kinderheilkunde und Jugendmedizin, Tübingen, Germany
  6. 6Department of Infectious Diseases and Immunology, Children's Hospital, University of Munich, Munich, Germany
  7. 7Department of General Internal Medicine, N4i Centre for Immunodeficiency and Autoinflammation (NCIA), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  8. 8Pediatria II, Reumatologia, Istituto G Gaslini, PRINTO Coordinating Centre, Genova, Italy
  9. 9Dipartimento di Scienze della Salute, Università di Genova, Genova, Italy
  10. 10Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy
  1. Correspondence to Dr Maryam Piram, Service de Pédiatrie Générale et Rhumatologie pédiatrique, Centre de référence des maladies auto-inflammatoires de l'enfant (CeRéMAI), CHU de Bicêtre, 78 Rue du Général Leclerc, Le Kremlin Bicêtre 94270, France; maryam.piram{at}


Objectives 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.

  • Fever Syndromes
  • Disease Activity
  • Familial Mediterranean Fever

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