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关节炎作为家族性地中海热的唯一发作性表现。

Arthritis as the sole episodic manifestation of familial Mediterranean fever.

作者信息

Lidar Merav, Kedem Ron, Mor Adam, Levartovsky David, Langevitz Pnina, Livneh Avi

机构信息

Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.

出版信息

J Rheumatol. 2005 May;32(5):859-62.

Abstract

OBJECTIVE

To clinically and genetically characterize patients with familial Mediterranean fever (FMF) in whom arthritis constitutes the only manifestation, and to establish the most important features distinguishing FMF arthritis in such a setting from other forms of mono/oligo arthritides.

METHODS

The study population comprised 14 patients with episodes of arthritis as the only manifestation of FMF who nevertheless fulfilled the diagnostic criteria for FMF. The control group consisted of 28 patients with episodic mono/oligo arthritis of different disease entities (palindromic, reactive, inflammatory bowel disease, Reiter's, seronegative spondyloarthropathy, chronic juvenile, Behcet's, and gouty arthritis) who presented to the rheumatology clinic during the study period. Patients in both groups underwent clinical evaluation and donated blood for FMF gene analysis.

RESULTS

The study and control groups shared similar age and sex distribution and experienced the monoarthritic attacks at similar sites, usually the knee and ankle joint. The 2 groups differed significantly in features of arthritis (which were febrile and of short duration in FMF), family history of FMF, mutation analysis, and response to colchicine. These differences allowed the defining of a rule, which readily distinguishes FMF arthritis from other forms of episodic mono/oligo arthritis.

CONCLUSION

The clinical, ethnic, and genetic features of recurrent monoarthritis of FMF are specific and may separate FMF from other entities with mono/oligo arthritis.

摘要

目的

对以关节炎为唯一表现的家族性地中海热(FMF)患者进行临床和基因特征分析,并确定在此种情况下FMF关节炎与其他形式的单关节炎/寡关节炎相区别的最重要特征。

方法

研究人群包括14例以关节炎发作为FMF唯一表现但仍符合FMF诊断标准的患者。对照组由28例在研究期间到风湿病门诊就诊的不同疾病实体(回纹型、反应性、炎症性肠病、赖特综合征、血清阴性脊柱关节病、慢性青少年型、白塞病和痛风性关节炎)的发作性单关节炎/寡关节炎患者组成。两组患者均接受临床评估并捐献血液用于FMF基因分析。

结果

研究组和对照组年龄和性别分布相似,单关节炎发作部位相似,通常为膝关节和踝关节。两组在关节炎特征(FMF中为发热且持续时间短)、FMF家族史、突变分析及对秋水仙碱的反应方面存在显著差异。这些差异使得能够确定一条规则,可轻易将FMF关节炎与其他形式的发作性单关节炎/寡关节炎区分开来。

结论

FMF复发性单关节炎的临床、种族和基因特征具有特异性,可能将FMF与其他单关节炎/寡关节炎实体区分开来。

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