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家族性地中海热患者的秋水仙碱反应能否预测?

Can colchicine response be predicted in familial Mediterranean fever patients?

机构信息

Division of Pediatric Rheumatology & Nephrology, Department of Pediatrics and Department of Biostatistics, Ankara University School of Medicine, Ankara, Turkey.

出版信息

Rheumatology (Oxford). 2014 Oct;53(10):1767-72. doi: 10.1093/rheumatology/keu138. Epub 2014 Apr 24.

Abstract

OBJECTIVES

The aims of this study were to explore whether the demographic and clinical features of paediatric familial Mediterranean fever (FMF) patients with different colchicine response vary or not and to determine whether colchicine response can be predicted in FMF patients.

METHODS

Files of patients who have been on colchicine therapy for at least 6 months were retrospectively evaluated. Patients were divided into two groups: group I included patients with no attacks after colchicine and group II comprised patients with ongoing attacks. Thereafter group II was further divided into two groups according to the reduction rate of attack frequency: group IIA (>50%) and group IIB (≤50%).

RESULTS

The study group comprised 221 FMF patients (116 females, 105 males). There were 131 patients in group I and 90 patients in group II (54 in group IIA and 36 in group IIB). Leg pain and M694V homozygosity were more frequent in group II (P < 0.05). Final colchicine doses, disease severity scores and number of patients with elevated acute phase reactant levels (attack-free period) were significantly higher and colchicine compliance was lower in group II when compared with group I (P < 0.05). Erysipelas-like erythema (ELE), leg pain and protracted arthritis/protracted febrile myalgia/vasculitis were more frequently detected in group IIB (P < 0.05).

CONCLUSION

Colchicine response is excellent in the majority of FMF patients, however, colchicine unresponsiveness cannot be predicted easily at onset. More rarely encountered clinical findings such as ELE, leg pain and protracted complaints and M694V homozygosity may be a clue for less colchicine response.

摘要

目的

本研究旨在探讨不同秋水仙碱反应的儿科家族性地中海热(FMF)患者的人口统计学和临床特征是否存在差异,并确定 FMF 患者的秋水仙碱反应是否可预测。

方法

回顾性评估接受秋水仙碱治疗至少 6 个月的患者的档案。患者被分为两组:组 I 包括秋水仙碱治疗后无发作的患者,组 II 包括持续发作的患者。此后,根据发作频率降低率将组 II 进一步分为两组:组 IIA(>50%)和组 IIB(≤50%)。

结果

研究组包括 221 例 FMF 患者(116 名女性,105 名男性)。组 I 中有 131 例患者,组 II 中有 90 例患者(54 例在组 IIA,36 例在组 IIB)。组 II 中腿部疼痛和 M694V 纯合子更为常见(P<0.05)。与组 I 相比,组 II 的最终秋水仙碱剂量、疾病严重程度评分和急性相反应物水平升高的患者数量(无发作期)更高,秋水仙碱依从性更低(P<0.05)。组 IIB 中更频繁地发现红斑样红斑(ELE)、腿部疼痛和迁延性关节炎/发热性肌痛/血管炎(P<0.05)。

结论

大多数 FMF 患者对秋水仙碱反应良好,但在发病时很难轻易预测秋水仙碱无反应。更罕见的临床发现,如 ELE、腿部疼痛和迁延性症状以及 M694V 纯合子,可能是秋水仙碱反应较差的线索。

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