肾动脉纤维肌性发育不良患者临床与血管造影特征的18年单中心病例总结

Clinical and Angiographic Characteristics of Patients With Renal Artery Fibromuscular Dysplasia:18 Years Data Analysis in a Single Medical Center

目的:旨在了解我国肾动脉纤维肌性发育不良(RA-FMD)患者的临床和血管造影特征。方法:回顾性连续收集中国医学科学院阜外医院2000年11月1日至2018年10月31日确诊RA-FMD的住院患者142例,分析其人口学、临床和血管造影资料,并与最近欧美纤维肌性发育不良注册数据库患者进行比较。结果:142例RA-FMD患者男女比例6:5,均罹患高血压。RA-FMD诊断年龄10~55(28.0±9.8)岁,高血压发病年龄6~54(23.9±9.1)岁。13例(9.2%)合并脑卒中,其中11例(7.8%)为脑梗死;62例(43.7%)合并肾脏损害,其中一侧肾萎缩49例(34.5%),25例(17.6%)尿蛋白阳性,7例(4.9%)肾功能不全,6例(4.2%)一侧肾梗死。肾动脉选择性造影或CT血管造影显示,共179条(63.0%)肾动脉受累,单右侧多见有69例(48.6%),局灶型多见有106例(74.6%)。肾动脉狭窄率为(85.2±12.7)%,闭塞24例(16.9%);狭窄主要分布在主干中段和远段,分别有53例(37.3%)和57例(40.1%);合并肾动脉夹层4例(2.8%);合并肾动脉瘤44例(31.0%),以单左侧多见,有28例(63.6%)。合并肾动脉外纤维肌性发育不良病变12例(8.5%)。与最近美国和欧洲纤维肌性发育不良患者相比,RA-FMD诊断年龄更小[(28.0±9.8)岁vs.(53.3±12.8)岁,(28.0±9.8)岁vs.(45.8±15.8)岁,P均<0.05],女性比例更低(46.5%vs.94.7%,46.5%vs.83.3%,P均<0.05),血管受累局灶型比例更高(74.6%vs.24.0%,74.6%vs.28.1%,P均<0.05),多系统血管受累比例更低(8.5%vs.55.1%,8.5%vs.31.2%,P均<0.05),至少1处动脉瘤比例较高(31.0%vs.22.7%,31.0%vs.20.0%,P均<0.05)。结论:中国RA-FMD患者发病年龄较小,男性多见,临床及血管造影特征与欧美人群有显著差异,需要进一步更大样本的中国多中心研究予以验证。

Objectives:To investigate the clinical and angiographic characteristics of patients with renal artery fibromuscular dysplasia(RA-FMD)hospitalized in Fuwai Hospital.Methods:Consecutive patients diagnosed with RA-FMD and hospitalized in Fuwai Hospital from November 1,2000 to October 31,2018 were enrolled in this retrospective analysis.The demography,clinical and angiographic characteristics were analyzed and compared with recent fibromuscular dysplasia(FMD)registration database in Europe and America.Results:During the study period,142 patients were diagnosed with RA-FMD(male and female ratio:6:5).The age at diagnosis was 10~55(28±9.8)years,and the onset age of hypertension was 6~54(23.9±9.1)years.All patients suffered from hypertension at admission,and 19(9.2%)patients suffered from stroke(including 11 cerebral infarction).Renal damages were evidenced in 62(43.7%)patients including 7(4.9%)renal insufficiency,25(17.6%)urinary protein,49(34.5%)renal atrophy,and 6(4.2%)renal infarction.A total of 179 renal arteries were involved.Sixty-nine(48.6%)patients had single right lesion and focal types were found in 106(74.6%)cases.Ratio of renal artery stenosis was 85.2%±12.7%,including 24(16.9%)cases of renal artery occlusion.Middle trunk renal artery stenosis was found in 53(37.3%)patients and distal trunk renal artery stenosis was evidenced in 57(40.1%)patients.Renal artery dissection was found in 4(16.9%)cases.Fortyfour(31.0%)cases had renal aneurysm,with 28(63.6%)single left aneurysms.Twelve(8.5%)cases were complicated with extrarenal lesions.Compared with recent FMD registration database in Europe and America,significant difference was found in most indexes,such as age at diagnosis of RA-FMD is younger,the proportion of women is lower,the proportion of focal types is higher,the proportion of multi-vascular involvement is lower,and the proportion of at least one aneurysm is higher(all P<0.05)in our cohort.Conclusions:Chinese patients with RA-FMD are younger and percent of male is higher,and the clinical and angiographic characteristics of these patients are significantly different from the report on European and American populations with FMD.Further multicenter studies with larger samples are needed in China.