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Cryopyrin-Associated Periodic Syndromes (CAPS)

  • Marinka TwiltEmail author
  • Susanne M. Benseler
Chapter

Abstract

Cryopyrin-associated periodic syndrome (CAPS) is an autoinflammatory disease spectrum due to autosomal dominant mutations in the NLRP3 gene resulting in excess secretion of interleukin 1. CAPS is characterized by systemic and organ inflammation baring the risk of irreversible damage including sensorineural hearing loss, renal failure, hydrocephalus, and skeletal deformities. International efforts have advanced our understanding of the disease mechanisms. Through partnerships we dramatically increased our ability to rapidly diagnose CAPS, select optimal therapies, monitor disease activity, and prevent damage accrual.

Keywords

Autoinflammation Inflammasome Cryopyrin NLRP3 Cryopyrin-associated periodic syndrome – CAPS Familial cold autoinflammatory syndrome – FCAS Muckle-Wells syndrome – MWS Neonatal-onset multisystem inflammatory disorder – NOMID Chronic infantile neurologic, cutaneous and articular – CINCA – syndrome Sensorineural hearing loss Interleukin 1 

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Alberta Children’s HospitalCalgaryCanada
  2. 2.Department of PaediatricsAlberta Children’s HospitalCalgaryCanada
  3. 3.Cumming School of MedicineUniversity of CalgaryCalgaryCanada

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