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停止儿童家族性地中海热的秋水仙碱治疗。

Discontinuation of Colchicine Therapy in Children With Familial Mediterranean Fever.

作者信息

Yonatan Butbul Aviel, Silman Rawan, Shafe Fahoum, Isaiah Wexler, Yackov Berkun

机构信息

Y. Butbul Aviel, MD, Department of Pediatrics B, Ruth Rappaport Children's Hospital, Rambam Health Care Campus, and Pediatric Rheumatology Service, Ruth Rappaport Children's Hospital, Rambam Health Care Campus, and The Ruth and Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa;

S. Rawan, MD, The Ruth and Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa.

出版信息

J Rheumatol. 2021 Nov;48(11):1732-1735. doi: 10.3899/jrheum.201158. Epub 2021 May 15.

DOI:10.3899/jrheum.201158
PMID:33993110
Abstract

OBJECTIVE

Colchicine has been considered a lifelong therapy for familial Mediterranean fever (FMF). Recent studies describe patients who discontinued colchicine, but there is a lack of data pertaining to predictors of success. The aims of our study were to describe a cohort of pediatric patients with FMF who discontinued colchicine therapy, and to identify factors predicting successful termination of colchicine.

METHODS

This study describes a cohort of pediatric patients with FMF who discontinued colchicine therapy following a relatively prolonged attack-free period (> 6 months), and identifies factors predicting successful termination. Data collected included demographic, clinical, and laboratory characteristics of children diagnosed with FMF aged < 16 years who underwent a trial of colchicine discontinuation. Data from patients who successfully ceased colchicine therapy were compared to those of patients who relapsed.

RESULTS

Of 571 patients with FMF, 59 (10.3%) discontinued colchicine therapy. The average attack-free period before enrollment was 0.97 ± 1.4 years. Follow-up after ceasing colchicine was 5.0 ± 3.05 years, during which time 11 (20%) patients had an attack. The most common symptoms were fever (100%) and abdominal pain (80%). For those failing discontinuation, colchicine was restarted within 1.3 years (range 0.3-5.0, median 0.7 yrs). A longer attack-free period prior to colchicine discontinuation predicted success. Myalgia and arthritis prior to colchicine cessation were more common among children who required renewal of colchicine.

CONCLUSION

Cessation of colchicine therapy should be considered following prolonged remission in a select group of patients. Patients with arthritis or myalgia are more likely to have an attack after ceasing colchicine therapy.

摘要

目的

秋水仙碱被认为是家族性地中海热(FMF)的终身治疗方法。最近的研究描述了停止使用秋水仙碱的患者,但缺乏成功预测指标的数据。我们的研究目的是描述一组停止秋水仙碱治疗的 FMF 儿科患者,并确定预测成功停药的因素。

方法

本研究描述了一组在相对较长的无发作期(> 6 个月)后停止秋水仙碱治疗的 FMF 儿科患者,并确定了成功停药的预测因素。收集的数据包括接受秋水仙碱停药试验的年龄<16 岁的 FMF 儿童的人口统计学、临床和实验室特征。成功停止秋水仙碱治疗的患者的数据与复发患者进行比较。

结果

在 571 例 FMF 患者中,59 例(10.3%)停止了秋水仙碱治疗。入组前的平均无发作期为 0.97 ± 1.4 年。停止秋水仙碱治疗后的随访时间为 5.0 ± 3.05 年,在此期间有 11 例(20%)患者出现发作。最常见的症状是发热(100%)和腹痛(80%)。对于那些不能停药的患者,秋水仙碱在停药后 1.3 年内(范围 0.3-5.0,中位数 0.7 年)重新开始使用。在停止秋水仙碱之前更长的无发作期预测了成功。在停止秋水仙碱之前有肌痛和关节炎的儿童更有可能在停止秋水仙碱治疗后发作。

结论

在选择的患者群体中,在长时间缓解后应考虑停止秋水仙碱治疗。有肌痛或关节炎的患者在停止秋水仙碱治疗后更有可能发作。

相关文献

1Discontinuation of Colchicine Therapy in Children With Familial Mediterranean Fever.PubMed译停止儿童家族性地中海热的秋水仙碱治疗。

Yonatan Butbul Aviel, Silman Rawan, Shafe Fahoum, et al.
J Rheumatol. 2021 Nov;48(11):1732-1735. doi: 10.3899/jrheum.201158. Epub 2021 May 15.

2Discontinuing colchicine in symptomatic carriers for MEFV (Mediterranean FeVer) variants.PubMed译对携带MEFV(地中海热)变异基因的有症状患者停用秋水仙碱。

et al.
Clin Rheumatol. 2017 Feb;36(2):421-425. doi: 10.1007/s10067-016-3421-8. Epub 2016 Sep 27.

3Familial Mediterranean fever in childhood: a single-center experience.PubMed译儿童期家族性地中海热:单中心经验。

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4Colchicine treatment can be discontinued in a selected group of pediatric FMF patients.PubMed译秋水仙碱治疗可在选择的一组儿科 FMF 患者中停药。

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5Effectiveness of Canakinumab in Colchicine- and Anakinra-Resistant or -Intolerant Adult Familial Mediterranean Fever Patients: A Single-Center Real-Life Study.PubMed译Canakinumab 在秋水仙碱和阿那白滞素耐药或不耐受的成年家族性地中海热患者中的疗效:一项单中心真实世界研究。

Hakan Babaoglu, Ozkan Varan, Hamit Kucuk, et al.
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6Successful management of colchicine resistant familial Mediterranean fever patients with a standardized canakinumab treatment protocol: a case series and literature review.PubMed译成功管理对秋水仙碱耐药的家族性地中海热患者的标准化康纳单抗治疗方案:病例系列和文献复习。

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7A score for predicting colchicine resistance at the time of diagnosis in familial Mediterranean fever: data from the TURPAID registry.PubMed译预测家族性地中海热患者诊断时对秋水仙碱耐药的评分:来自 TURPAID 登记处的数据。

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8Treatment of colchicine-resistant Familial Mediterranean fever in children and adolescents.PubMed译儿童和青少年中对秋水仙碱耐药的家族性地中海热的治疗。

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10Do all colchicine preparations have the same effectiveness in patients with familial Mediterranean fever?PubMed译对于家族性地中海热患者,所有秋水仙碱制剂的疗效都相同吗?

Mod Rheumatol. 2021 Mar;31(2):481-484. doi: 10.1080/14397595.2020.1790139. Epub 2020 Jul 20.

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