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Results Fourteen French patients were identified (6 women/8 men) including MWS (n=9), FCAS (n=3), CINCA (n=2) and having received interleukin-1 inhibitors in 7 cases. Mean age at the diagnosis of amyloidosis was 22.6 years and five (35.7 %) patients died. We found 53 patients in the literature, with a sex ratio of 1. They included MWS (n=34), FCAS/MWS (n=12) and FCAS (n=7). Among 67 patients (French and literature), the median age at amyloidosis diagnosis was 30 years, ranging from 12 to 61 years. The NLRP3 gene was sequenced in 30 patients (45.5%), and the distribution of amino acids changes was as follows: R262W (n=16), T348M (n=5), A439V (n=4), D303N (n=3), T436N (n=2) and L353P (n=1). 23 patients had died (35%), but none of them had received interleukin-1 inhibitors. Since 2002, 24/67 (36%) patients with CAPS-associated amyloidosis have received interleukin-1 inhibitors, with at least a decrease of proteinuria and creatininemia in 9 of them (37.5%)

pediatric rheumatology online journal

French Amyloidosis CAPS study: AA Amyloidosis complicating cryopyrin-associated periodic syndrome: a study on 14 cases and review of 53 cases from literature