Article Text

Download PDFPDF
Extended report
A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference
  1. Maryam Piram1,
  2. Joost Frenkel2,
  3. Marco Gattorno3,
  4. Seza Ozen4,
  5. Helen J Lachmann5,
  6. Raphaela Goldbach-Mansky6,
  7. Véronique Hentgen7,
  8. Bénédicte Neven8,
  9. Katia Stankovic Stojanovic9,
  10. Anna Simon10,
  11. Jasmin Kuemmerle-Deschner11,
  12. Hal Hoffman12,
  13. Silvia Stojanov13,
  14. Agnès Duquesne14,
  15. Pascal Pillet15,
  16. Alberto Martini3,
  17. Jacques Pouchot16,
  18. Isabelle Koné-Paut1
  19. on behalf of the EUROFEVER and EUROTRAPS networks
  1. 1Department of Pediatrics and Pediatric Rheumatology, National Reference Center for Auto-Inflammatory Disorders, Bicêtre University Hospital, Paris XI University, Le Kremlin-Bicêtre, France
  2. 2Department of Pediatrics, University Medical Center Utrecht, Utrtecht, The Netherlands
  3. 3UO Pediatria II and Department of Pediatrics, “G Gaslini” Institute and University of Genoa, Italy
  4. 4Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey
  5. 5National Amyloidosis Centre, University College London Medical School, London, UK
  6. 6National Institute of Arthritis Musculoskeletal and Skin Disease, NIH, Bethesda, Maryland, USA
  7. 7Department of Pediatrics, National Reference Center for Auto-Inflammatory Disorders, A Mignot Hospital, Versailles, France
  8. 8Department of Pediatric Immuno-Hematology and Pediatric Rheumatology, Necker-Enfants Malades Hospital, Paris, France
  9. 9Department of Internal Medicine, National Reference Center for Inflammatory Amyloidosis and Familial Mediterranean Fever, Tenon University Hospital, Université Pierre et Marie Curie Paris 6, Paris, France
  10. 10Department of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  11. 11Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Germany
  12. 12Department of Pediatrics and Medicine, University of California, San Diego, Rady Children's Hospital of San Diego, San Diego, California, USA
  13. 13Department of Infectious Diseases and Immunology, University Children's Hospital, Munich, Germany
  14. 14Department of Nephrology and Pediatric Rheumatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France
  15. 15Department of Pediatrics, Children's Hospital of Bordeaux, France
  16. 16Department of Internal Medicine, European Georges Pompidou Hospital, Paris-Descartes University, Paris, France
  1. Correspondence to Professor Isabelle Koné-Paut, Department of Pediatrics and Pediatric Rheumatology, National Reference Center for Auto-Inflammatory Disorders, Bicêtre University Hospital, Paris XI University, 78 rue du Général Leclerc, 94270 Le Kremlin Bicêtre, France; isabelle.kone-paut{at}


Background The systemic autoinflammatory disorders (SAID) share many clinical manifestations, albeit with variable patterns, intensity and frequency. A common definition of disease activity would be rational and useful in the management of these lifelong diseases. Moreover, standardised disease activity scores are required for the assessment of new therapies in constant development. The aim of this study was to develop preliminary activity scores for familial Mediterranean fever, mevalonate kinase deficiency, tumour necrosis factor receptor-1-associated periodic syndrome and cryopyrin-associated periodic syndromes (CAPS).

Methods The study was conducted using two well-recognised consensus formation methods: the Delphi technique and the nominal group technique. The results from a two-step survey and data from parent/patient interviews were used as preliminary data to develop the agenda for a consensus conference to build a provisional scoring system.

Results 24 of 65 experts in SAID from 20 countries answered the web questionnaire and 16 attended the consensus conference. There was consensus agreement to develop separate activity scores for each disease but with a common format based on patient diaries. Fever and disease-specific clinical variables were scored according to their severity. A final score was generated by summing the score of all the variables divided by the number of days over which the diary was completed. Scores varied from 0 to 16 (0–13 in CAPS). These scores were developed for the purpose of clinical studies but could be used in clinical practice.

Conclusion Using widely recognised consensus formation techniques, preliminary scores were obtained to measure disease activity in four main SAID. Further prospective validation study of this instrument will follow.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Funding sources This study was supported by grants from the French Ministry of Health (PHRC 2008) and Assistance Publique-Hôpitaux de Paris (APHP).

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.