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Recommendations for the management of autoinflammatory diseases


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.

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ter Haar, N., Oswald, M., Jeyaratnam, J. et al. Recommendations for the management of autoinflammatory diseases. Pediatr Rheumatol 13 (Suppl 1), P133 (2015).

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