TY - JOUR T1 - Longterm Followup of Quality of Life in Patients with Cryopyrin-associated Periodic Syndrome Treated with Canakinumab, an Anti-interleukin 1β Monoclonal Antibody JF - The Journal of Rheumatology JO - J Rheumatol SP - 1721 LP - 1722 DO - 10.3899/jrheum.131291 VL - 41 IS - 8 AU - CÉLINE MARSAUD AU - ISABELLE MARIE AU - ISABELLE KONÉ-PAUT Y1 - 2014/08/01 UR - http://www.jrheum.org/content/41/8/1721.abstract N2 - To the Editor:Cryopyrin-associated periodic syndrome (CAPS) is an inherited autoinflammatory disorder that comprises a clinical spectrum of 3 distinct phenotypes increasing in severity: familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and chronic infantile neurological cutaneous and articular syndrome (CINCA). Urticarial rash, fever, arthralgia, and intense fatigue are the main clinical signs1. CINCA is the most severe form, with mental and physical disability. Some patients are difficult to classify because they present intermediate phenotypes. The effect of CAPS on quality of life is significant in all phenotypes, with limitation in ability to work (78%) and in participation in outdoor activities (95%)2.The NOD-like receptor 3 (NLPR3) gene mutation is responsible for overproduction of interleukin 1β (IL-1β)3. Three IL-1 inhibitors have shown efficacy in CAPS: anakinra, rilonacept, and canakinumab4. The approval of canakinumab by the US Food and Drug Administration was based on a 48-week, double-blinded study showing rapid and sustained remission of symptoms under treatment5.Our objective with this monocentric longitudinal study was to evaluate quality of life and socioprofessional effects of … Address correspondence to Dr. C. Marsaud, Department of Pediatrics and Pediatric Rheumatology, Bicêtre University Hospital, Paris XI University, 78 rue du Général Leclerc, 94270 Le Kremlin Bicêtre, Paris, France. E-mail: celine.marsaud{at}bct.aphp.fr ER -