多发性大动脉炎125例临床分析

Retrospective study of 125 patients with Takayasu arteritis

目的:探讨成人及青少年多发性大动脉炎(Takayasu arteritis,TA)的临床、影像学特征,分析相关治疗方法及转归。方法:回顾性分析125例TA患者的临床资料。结果:125例患者中女性108例,男性17例;平均发病年龄为(26.9±11.0)岁,31例起病时为青少年(≤18岁)。38.4%的患者有发热、关节痛等全身症状,71.2%的患者具有无脉或脉弱,红细胞沉降率(ESR)增高见于49.6%的患者。与成人患者相比,青少年中全身症状、脉搏减弱及ESR增高较为少见(P<0.05)。动脉造影结果显示Ⅰ型(40%)、Ⅳ型(20.8%)和Ⅴ型(30.4%)多见,而Ⅱa型(4.8%)、Ⅱb型(1.6%)和Ⅲ型(2.4%)少见。成人患者中Ⅰ型TA明显高于青少年患者(P<0.05)。有7/12例患者临床表现为静止期,但血管病理显示有炎细胞浸润。80例接受随访,随访中位时间为36个月。随访中58例接受糖皮质激素治疗,16例需联合细胞毒药物治疗;57例经治疗病情得到缓解。36例行血管旁路移植术治疗,21例行血管内介入治疗,再狭窄率分别为34.7%及77.3%。结论:与成人TA患者相比,青少年患者中无脉症少见。临床表现为静止期的患者仍可能有活动性血管病变。血管内介入治疗与外科手术相比再狭窄发生率较高。

Objective：To study the clinical features,image findings,therapeutic strategies,and prognosis of adult and juvenile patients with Takayasu arteritis （TA）.Methods： The clinical data of 125 patients with TA were retrospectively studied.Results： There were 108 female and 17 male patients.The mean age of onset was （26.9±11.0） years and 31 patients were younger than 18 years at onset.Constitutional symptoms,pulse deficit,and elevated erythrocyte sedimentation rate （ESR） were present in 38.4%,71.2%,and 49.6% of patients,respectively.Compared with adult patients,the incidences of constitutional symptoms,pulse deficit and elevated ESR were lower in juveniles（P〈0.05）.Angiographic findings showed that Type Ⅰ（40%）,Type Ⅳ（20.8%） and Type Ⅴ（30.4%） were common,and Type Ⅱa （4.8%）,Type Ⅱb （1.6%） and Type Ⅲ（2.4%） were seldom seen.Type Ⅰ was more frequently seen in adult patients than in juveniles（P〈0.05）.Biopsy findings of 12 patients with quiescent disease showed active lesions in 7 patients.Eighty patients were followed up for a median duration of 36 months.Glucocorticoid was prescribed to 58 patients; cytotoxic agents were required for 16 of them; and remission was achieved in 57 patients.Bypass and endovascular interventional procedures were performed in 36 and 21 patients,respectively.Restenosis occurred in 34.7% of bypass procedures and 77.3% of endovascular interventional procedures.Conclusion： Diminished pulse is rarely seen in juvenile TA patients than in adults.Active vascular disorders may exist in patients with stable clinical manifestations.The incidence of restenosis is higher in endovascular interventional procedures than in bypass procedures.