title: Current treatment recommendations and considerations for cryopyrin-associated periodic syndrome.
abstract: Cryopyrin-associated periodic syndrome (CAPS) encompasses a spectrum of three phenotypes of increasing severity. The syndrome is due to dominant mutations in NLRP3, which encodes a key component of the innate immunity that regulates the secretion of IL-1β. CAPS manifests as systemic inflammation, which compromises quality of life and leads to serious complications and handicap. Anti-IL-1 drugs have shown remarkable efficacy in treating CAPS symptoms and have significantly changed patients' lives. They have acceptable safety profiles but do have some differences. We review three drugs that are currently marketed for CAPS, give additional information for the practical use of these drugs, and provide some recommendations for management.
authors: Isabelle
authors: I
authors: Koné-Paut
authors: a Division of Pediatric Rheumatology, Reference Center for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Le Kremlin Bicêtre Cedex, France.
authors: Caroline
authors: C
authors: Galeotti
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D002352:Carrier Proteins / Q000235:genetics*; D056587:Cryopyrin-Associated Periodic Syndromes / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D007113:Immunity, Innate / Q000235:genetics; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D053583:Interleukin-1beta / Q000037:antagonists & inhibitors / Q000276:immunology*; D009154:Mutation / Q000235:genetics; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D002352:Carrier Proteins; D053590:Interleukin 1 Receptor Antagonist Protein; D053583:Interleukin-1beta; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human; D017472:Receptors, Interleukin-1; D011993:Recombinant Fusion Proteins; C541220:canakinumab; C531377:rilonacept
keywords: Muckle–Wells syndrome; anakinra; autoinflammatory diseases; canakinumab; chronic infantile neurological cutaneous and articular syndrome; cryopyrin-associated periodic syndrome; familial cold autoinflammatory syndrome; interleukin 1; neonatal-onset multisystem inflammatory disease (NOMID); recommendations; rilonacept; treatment