误诊为大动脉炎的纤维肌性发育不良一例分析及文献复习

Fibromuscular dysplasia misdiagnosed as takayasu arteritis: a case report and literature review

目的提高风湿科医生对纤维肌性发育不良的认识。方法报告一例被误诊为大动脉炎的纤维肌性发育不良病例并复习相关文献。结果患者为16岁女性,急性起病,以高血压脑病为主要临床表现,所有免疫指标均阴性,多次查血沉、C反应蛋白等炎性指标均正常,血管造影显示右肾动脉主干远段狭窄,狭窄段呈串珠样改变,证实纤维肌性发育不良的诊断。球囊扩张术后临床症状消失,血压恢复正常,随诊半年病情稳定。结论纤维肌性发育不良是一种动脉病,因其发病的性别年龄特点及病变分布与大动脉炎相似,故临床上易与大动脉炎相混淆,但纤维肌性发育不良无特异性的炎性表现,血管造影呈典型的串珠样改变,累及肾动脉时位于肾动脉主干的远段而区别于大动脉炎,风湿科医生应予重视。

Objective To improve the rheumatologists＇ understanding of fibromuscular dysplasia. Methods A case of fibromuscular dysplasia that was misdiagnosed as Takayasu arteritis was reported and the related literatures were reviewed. Result A 16-year-old female presented clinically with an acute onset of hypertensive encephalopathy. All the immunological parameters were normal, and erythrocyte sedimentation rate （ESR）, C reactive protein （CRP） were normal repeatively. The angiography showed stenosis of the distal main trunk of the right renal artery and ＂.strings of beads＂ appearance, which pointed to the diagnosis of fibromuscular dysplasia. The clinical symptoms were relieved after using balloon dilator, and the blood pressure returned to normal. Her condition remained stable after half a year follow-up. Conclusion Fibromuscular dysplasia is an non athero-disease, Rheumatologists should pay attention to it in the differential diagnosis because fibremuselar dysplasia and Takayasu arteritis have the similar age, gender and involvement preferences. Fibromuscular dysplasia has no specific inflammatory manifestations, and often affects the distal main trunk of the renal artery and shows typical ＂strings of beads＂ appearance, which is different from takayasu artertis.