%0 Journal Article
%T Canakinumab (ACZ885, a fully human IgG1 anti-IL-1¦Â mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS)
%A Jasmin B Kuemmerle-Deschner
%A Eduardo Ramos
%A Norbert Blank
%A Joachim Roesler
%A Sandra D Felix
%A Thomas Jung
%A Kirstin Stricker
%A Abhijit Chakraborty
%A Stacey Tannenbaum
%A Andrew M Wright
%A Christiane Rordorf
%J Arthritis Research & Therapy
%D 2011
%I BioMed Central
%R 10.1186/ar3266
%X Seven pediatric patients (five children and two adolescents) with CAPS were enrolled in a phase II, open-label study of canakinumab in patients with CAPS. Canakinumab was administered at a dose of 2 mg/kg subcutaneously (s.c.) (for patients with body weight ¡Ü 40 kg) or 150 mg s.c. (for patients with body weight > 40 kg) with re-dosing upon each relapse. The primary efficacy variable was time to relapse following achievement of a complete response (defined as a global assessment of no or minimal disease activity and no or minimal rash and values for serum C-reactive protein (CRP) and/or serum amyloid A (SAA) within the normal range, < 10 mg/L).All patients achieved a complete response within seven days after the first dose of canakinumab and responses were reinduced on retreatment following relapse. Improvements in symptoms were evident within 24 hours after the first dose, according to physician assessments. The estimated median time to relapse was 49 days (95% CI 29 to 68) in children who received a dose of 2 mg/kg. Canakinumab was well tolerated. One serious adverse event, vertigo, was reported, but resolved during treatment.Canakinumab, 2 mg/kg or 150 mg s.c., induced rapid and sustained clinical and biochemical responses in pediatric patients with CAPS.ClinicalTrials.gov: NCT00487708Cryopyrin-associated periodic syndrome (CAPS) comprises a spectrum of rare inherited chronic auto-inflammatory disorders including familial cold auto-inflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), neonatal onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurological, cutaneous, and articular syndrome (CINCA). Common characteristics of these disorders include high-grade fever, urticarial rash, ocular manifestations such as conjunctivitis, sensorineural hearing loss and arthritis [1-5].Onset of symptoms generally occurs early in life, especially in patients with the two more severe phenotypes, MWS, and NOMID, and these disorders are associate
%U http://arthritis-research.com/content/13/1/R34