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  • Open Access

Recommendations for the management of autoinflammatory diseases

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Pediatric Rheumatology201513 (Suppl 1) :P133

  • Published:


  • Rheumatic Disease
  • Tumor Necrosis Factor Receptor
  • Autoinflammatory Disease
  • Factor Receptor Associate
  • Nominal Group Technique


Autoinflammatory diseases are rare disorders that lead to significant morbidity and mortality. Due to the low patient numbers, evidence-based guidelines are lacking and management is mostly based on physician's experience. In 2012, a European initiative called SHARE was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. One of the aims of SHARE was to provide evidence-based recommendations for the management of the autoinflammatory diseases Cryopyrin-Associated Periodic Syndromes (CAPS), Tumor necrosis factor Receptor Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency (MKD).


Evidence-based recommendations were developed using the European League Against Rheumatism (EULAR) 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, six recommendations on diagnosis, twenty recommendations on therapy and twelve recommendations on monitoring were accepted with ≥80% agreement among the experts. Topics include (but are not limited to) the use of validated scores for diagnosis and disease activity, therapy with biologicals, NSAIDs and corticosteroids, and items to assess in monitoring of a patient.


The SHARE initiative provides recommendations for the management of the autoinflammatory diseases CAPS, TRAPS and MKD.

Authors’ Affiliations

University Medical Center Utrecht, Utrecht, Netherlands
University Hospital Tuebingen, Tuebingen, Germany
Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain
National Institute of Health, Bethesda, USA
UCL Institute of Child Health, London, UK
Policlinico Le Scotte, University of Siena, Siena, Italy
Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, Paris, France
Hôpital Tenon, University Pierre-et-Marie-Curie, Paris, France
French Reference Centre for Auto-Inflammatory Diseases in Children, Centre Hospitalier de Versailles, Le Chesnay Cedex, France
University of Lausanne, Lausanne, Switzerland
Charité University Medicine, Berlin, Germany
UCL Medical School, London, UK
Cerrahpasa Ic Hastaliklari Klinigi, Universtity Istanbul, Istanbul, Turkey
Hacettepe University Faculty of Medicine, Ankara, Turkey
Hospital de Pediatría Garrahan, Buenos Aires, Argentina
Radboud University Medical Center, Nijmegen, Netherlands
Meir Medical Center, Tel-Aviv University, Tel-Aviv, Israel
University Hospital Leuven, Leuven, Belgium
The Hospital for Sick Children, Toronto, Canada
Alberta Children's Hospital, University of Calgary, Calgary, Canada
G. Gaslini Institute, Genoa, Italy


© ter Haar et al. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.