Abstract

Cryopyrin-associated periodic syndromes (CAPS) is a group of rare monogenetic autoinflammatory disorders. Evidence-based guidelines are lacking and management is mostly based on physician’s experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases.

Highlights

  • Cryopyrin-associated periodic syndromes (CAPS) is a group of rare monogenetic autoinflammatory disorders

  • Recommendations derived from the literature were evaluated by an online survey

  • Fifteen recommendations were suggested in the online survey and discussed during the consensus meeting

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Summary

Introduction

Cryopyrin-associated periodic syndromes (CAPS) is a group of rare monogenetic autoinflammatory disorders. Evidence-based guidelines are lacking and management is mostly based on physician’s experience. In 2012, a European initiative called SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases

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