Perioperative Management of Cryopyrin-Associated Periodic Syndrome, Muckle-Wells Subtype: A Case Report

Cryopyrin-associated periodic syndrome (CAPS) is a rare family of autoinflammatory disorders characterized by constitutive inflammasome activation that can be triggered by exposure to cold temperatures. We discuss the case of a 49-year-old woman with Muckle-Wells syndrome, a subtype of CAPS, presenting for cervical lymph node biopsy to rule out lymphoma. The pathophysiology of CAPS, intraoperative temperature monitoring, and other anesthesia considerations are reviewed in this article.