title: Impact of IL-1 inhibition on fatigue associated with autoinflammatory syndromes.
abstract: Cryopyrin-associated periodic syndromes (CAPS) is a rare group of autoinflammatory disorders that includes familial cold autoinflammatory syndrome or FCAS, Muckle-wells syndrome or MWS, and neonatal-onset multisystem inflammatory disease or NOMID. CAPS is caused by a mutation in the NOD-like receptor family, pyrin domain containing 3 (NLRP3) gene. This ultimately leads to increased production of interleukin (IL)-1β. IL-1β is a biologically active member of the IL-1 family. It is not only a pro-inflammatory cytokine responsible for features such as fever, rash, and arthritis, but is also a major mediator in the central pathways of fatigue. Fatigue is a major component of CAPS and is associated with severely compromised quality of life. In clinical studies, fatigue was measured using functional assessment of chronic illness therapy-fatigue or FACIT-F and short form-36 or SF-36, physical component score instruments. These questionnaires can also be used to monitor improvement of fatigue following initiation of therapy. IL-1 inhibitors block the IL-1 signaling cascade, thereby preventing systemic inflammation in CAPS. The decrease in systemic inflammation is accompanied by improvement in fatigue.
authors.affiliation: a Associate chief, Rheumatology Division, New York Methodist Hospital , Brooklyn , NY , USA.
authors.forename: Sujani
authors.initials: S
authors.lastname: Yadlapati
authors.affiliation: b Rheumatology Division, New York Methodist Hospital, Associate Professor of Clinical Medicine and Rheumatology, Weill Cornell Medical College , New York , NY , USA.
authors.forename: Petros
authors.initials: P
authors.lastname: Efthimiou
mesh_terms: D002352:Carrier Proteins / Q000235:genetics*; D056587:Cryopyrin-Associated Periodic Syndromes / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D005221:Fatigue / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics; D006801:Humans; D053583:Interleukin-1beta / Q000037:antagonists & inhibitors*; D009154:Mutation; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D011788:Quality of Life
chemical_list: D002352:Carrier Proteins; D053583:Interleukin-1beta; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human
keywords: Autoinflammatory diseases; CAPS; Fatigue; IL-1 inhibitors; IL-1β