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Recommendations for the management of autoinflammatory diseases
  1. Nienke M ter Haar1,2,
  2. Marlen Oswald3,
  3. Jerold Jeyaratnam4,
  4. Jordi Anton5,
  5. Karyl S Barron6,
  6. Paul A Brogan7,
  7. Luca Cantarini8,
  8. Caroline Galeotti9,
  9. Gilles Grateau10,
  10. Veronique Hentgen11,
  11. Michael Hofer12,
  12. Tilmann Kallinich13,
  13. Isabelle Kone-Paut14,
  14. Helen J Lachmann15,
  15. Huri Ozdogan16,
  16. Seza Ozen17,
  17. Ricardo Russo18,
  18. Anna Simon19,
  19. Yosef Uziel20,
  20. Carine Wouters21,
  21. Brian M Feldman22,
  22. Sebastiaan J Vastert2,
  23. Nico M Wulffraat2,
  24. Susanne M Benseler23,
  25. Joost Frenkel4,
  26. Marco Gattorno24,
  27. Jasmin B Kuemmerle-Deschner3
  1. 1Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands
  2. 2Department of Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands
  3. 3Division of Paediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany
  4. 4Department of Paediatrics, University Medical Center Utrecht, Utrecht, The Netherlands
  5. 5Paediatric Rheumatology Unit, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain
  6. 6Division of Intramural Research, National Institute of Health, Bethesda, USA
  7. 7Department of Rheumatology, UCL Institute of Child Health, London, UK
  8. 8Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy
  9. 9Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, Paris, France
  10. 10Department of Internal Medicine, APHP, Hôpital Tenon, University Pierre-et-Marie-Curie, Paris, France
  11. 11French Reference Centre for Auto-Inflammatory Diseases in Children, Centre Hospitalier de Versailles, Le Chesnay Cedex, France
  12. 12Department of Paediatrics, University of Lausanne, Lausanne, Switzerland
  13. 13Department of Paediatric Pneumology and Immunology, Charité University Medicine, Berlin, Germany
  14. 14Department of Paediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France
  15. 15National Amyloidosis Centre, University College London Medical School, London, UK
  16. 16Division of Rheumatology, Cerrahpasa Ic Hastaliklari Klinigi, University Istanbul, Istanbul, Turkey
  17. 17Department of Paediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey
  18. 18Service of Immunology and Rheumatology, Hospital de Pediatría Garrahan, Buenos Aires, Argentina,
  19. 19Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands
  20. 20Department of Paediatrics, Meir Medical Center, Tel-Aviv University, Tel-Aviv, Israel
  21. 21Department of Microbiology and Immunology, University Hospital Leuven, Leuven, Belgium
  22. 22Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada
  23. 23Department of Paediatrics, Rheumatology, Alberta Children's Hospital, University of Calgary, Calgary, Canada
  24. 24Division of Paediatrics II, G. Gaslini Institute, Genoa, Italy
  1. Correspondence to Nienke ter Haar, Department of Paediatric Immunology, and Laboratory for Translational Immunology, University Medical Center Utrecht, Lundlaan 6, Utrecht 3584 EA, The Netherlands; n.m.terhaar-2{at}


Autoinflammatory diseases are characterised by fever and systemic inflammation, with potentially serious complications. Owing to the rarity of these diseases, evidence-based guidelines are lacking. In 2012, the European project Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate regimens for the management of children and young adults with rheumatic diseases, facilitating the clinical practice of paediatricians and (paediatric) rheumatologists. One of the aims of SHARE was to provide evidence-based recommendations for the management of the autoinflammatory diseases cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) and mevalonate kinase deficiency (MKD). These recommendations were developed using the European League Against Rheumatism standard operating procedure. An expert committee of paediatric and adult rheumatologists was convened. Recommendations derived from the systematic literature review were evaluated by an online survey and subsequently discussed at a consensus meeting using Nominal Group Technique. Recommendations were accepted if more than 80% agreement was reached. In total, four overarching principles, 20 recommendations on therapy and 14 recommendations on monitoring were accepted with ≥80% agreement among the experts. Topics included (but were not limited to) validated disease activity scores, therapy and items to assess in monitoring of a patient. By developing these recommendations, we aim to optimise the management of patients with CAPS, TRAPS and MKD.

  • Fever Syndromes
  • Treatment
  • Multidisciplinary team-care

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