作者: Alice Corsia , Sophie Georgin-Lavialle , Véronique Hentgen , Eric Hachulla , Gilles Grateau
DOI: 10.1186/S13023-017-0609-1
关键词:
摘要: Colchicine is the standard treatment for familial Mediterranean fever (FMF), preventing attacks and inflammatory complications. True resistance rare yet not clearly defined. We evaluated physicians’ definition of colchicine report how they manage it. recruited patients with a clinical diagnosis FMF, one exon-10 (MEFV) gene mutation considered resistant to colchicine, via networks expert physicians. Clinical, biological characteristics information about (dose adjustment, compliance) were collected. The severity FMF was assessed by Tel Hashomer criteria. included 51 patients, most females (55%), mean age 34 ± 23.1 years years (range 4.7–86.3). Overall, 58% (27/47) had homozygous M694 MEFV mutations. Seventeen 42 (40%) declared full adherence treatment, greater children (48%) than adults (22%). Physicians with > 6 attacks/year (n = 21/51, 42%), > 4 in last 6 months (n = 13/51, 26%), persistent inflammation (n = 23/51, 45%), renal amyloidosis (n = 6/28, 22%) adult intolerance an increase dose (n = 10/51, 19%), other reasons 23%), including chronic arthralgia (n = 6/51, 12%). Interleukin 1–targeting drugs represented only alternative treatments addition daily colchicine. Resistance (<10% patients) mostly observed severe genotypes. main physicians assessing symptoms, subclinical inflammation, secondary amyloidosis. Low key component resistance.