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Recommendations for the management of autoinflammatory diseases

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Background

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).

Methods

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.

Results

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.

Conclusion

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

Author information

Correspondence to N ter Haar.

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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 (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Keywords

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