最多等待 10 秒，若超时请稍后重试。
Assistance publique-hôpitaux de Paris, hôpital Tenon, Centre de référence adulte de la fièvre méditerranéenne familiale, service de médecine interne, F-75020 Paris, France; Sorbonne Universités, UPMC Université Paris 06, Département hospitalo-universitaire I2B, F-75013 Paris, France.
Centre hospitalier de Versailles, centre de référence des maladies autoinflammatoires rares de l'enfant (CeRéMAI), service de pédiatrie, F-78150 Le Chesnay, France.
Clin Biochem. 2017 Mar;50(4-5):206-209. doi: 10.1016/j.clinbiochem.2016.11.008. Epub 2016 Nov 10.
Monitoring SAA level in attack-free FMF patients is recommended in order to adjust colchicine dose, and minimize the risk of AA amyloidosis. In countries where this test is not available, C-reactive protein (CRP), another acute phase reactant, is used instead. However, CRP is low and SAA is increased in some patients and vice versa.
To determine the threshold of CRP corresponding to SAA<10mg/L in patients with FMF and to assess their concordance at the patient level.
Consecutive FMF patients in attack-free period and no other cause of intermittent inflammation including infections were recruited during their regular visits in the French reference center for FMF. Demographic and genetic data were recorded; CRP and SAA were tested simultaneously. The threshold value of CRP corresponding to 10mg/L for SAA was determined and the concordance between the two markers was assessed with Cohen's kappa index.
399 samples were obtained from 218 patients, mean age of 27years (33% under 18years old), 55% of female, from Sephardic Jewish origin in 71%. MEFV mutation was M694V homozygous or compound heterozygous in 52%, and simple heterozygous in 18%. Six patients had AA amyloidosis. The appropriate CRP threshold was found to be 5mg/L in children and 8.75mg/L in adults. Global agreement with SAA<10mg/L was 84% [95% confidence interval: 82 to 86%], leading to a kappa index at 0.62 [95% confidence interval: 0.57 to 0.68].
CRP<5mg/L in FMF children or 8.75mg/L in FMF adults during attack-free periods might be a convenient substitute to guide therapeutic decisions when SAA is unavailable.
建议对无发作的家族性地中海热（FMF）患者监测血清淀粉样蛋白A（SAA）水平，以调整秋水仙碱剂量，并将继发性淀粉样变性病（AA）的风险降至最低。在无法进行该检测的国家，则使用另一种急性期反应物C反应蛋白（CRP）来替代。然而，部分患者CRP水平较低而SAA升高，反之亦然。
确定FMF患者中与SAA<10mg/L相对应的CRP阈值，并在患者层面评估二者的一致性。
在法国FMF参考中心，连续纳入处于无发作期且无包括感染在内的其他间歇性炎症病因的FMF患者。记录人口统计学和遗传学数据；同时检测CRP和SAA。确定与SAA为10mg/L相对应的CRP阈值，并使用科恩kappa指数评估这两种标志物之间的一致性。
从218例患者中获取了399份样本，平均年龄27岁（33%年龄在18岁以下），55%为女性，71%为西班牙裔犹太人。52%的患者MEFV突变是M694V纯合子或复合杂合子，18%为单纯杂合子。6例患者患有AA淀粉样变性病。发现儿童的适宜CRP阈值为5mg/L，成人为8.75mg/L。与SAA<10mg/L的总体一致性为84%[95%置信区间：82%至86%]，kappa指数为0.62[95%置信区间：0.57至0.68]。
在无发作期，FMF儿童CRP<5mg/L或FMF成人CRP<8.75mg/L可能是在无法检测SAA时指导治疗决策的便捷替代指标。
