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Criteria for CAPS, is it all in the name?
  1. Isabelle Touitou,
  2. Guillaume Sarrabay
  1. Reference Centre for Autoinflammatory Diseases, Hôpital A de Villeneuve, CHRU Montpellier, Montpellier, France
  1. Correspondence to Professor Isabelle Touitou, Reference Centre for Autoinflammatory Diseases, Hôpital A de Villeneuve, CHRU Montpellier, 34295 Montpellier Cedex 5, France; Isabelle.touitou{at}inserm.fr

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This paper1 describes an original work conducted by an International team of 16 recognised clinical experts in the field of autoinflammatory diseases. The aim of this consortium was to develop diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS). They resulted in a model that indisputably is relevant to describe these rare and heterogeneous diseases among other autoinflammatory diseases. Their proposed CAPS diagnosis criteria are primarily clinical.

We would like to comment on the pathophysiological mechanism underlying ‘CAPS’. The NLRP3 gene encodes cryopyrin, the historical name of the …

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