多发性大动脉炎67例诊治分析

Retrospective Study of 67Patients with Takayasu Arteritis

目的：探究多发性大动脉炎（Takayasu arteritis,TA）患者的临床疾病谱、特征及治疗转归。方法：回顾分析67例多发性大动脉炎患者的临床资料。结果：67例患者中,男女比例为1∶4.6,平均年龄为35岁（15-74岁）,病程0-32年。患者常见的临床症状包括乏力（56.7%）、头晕或头痛（44.8%）、胸闷或胸痛（41.8%）、上肢血压不对称（35.8%）,其次为肢体发冷（11.9%）、肢端麻木（13.4%）、间歇性跛行（9.0%）等。影像学检查发现,IV型最多（31.3%）,其次为I型（19.4%）,且患者的疾病活动性与影像学表现存在相关性。所有患者中,3例患者病情稳定,未予特殊治疗;45例接受糖皮质激素治疗;46例接受环磷酰胺（cyclophosphamide,CTX）治疗,有效36例（78%）,平均CTX累积剂量为3.43 g;CTX治疗无效者改用硫唑嘌呤（5例,其中1例同时合用来氟米特）、甲氨蝶呤（2例）、肿瘤坏死因子拮抗剂（2例）,1例患者改用秋水仙碱加来氟米特,治疗后患者病情均得到缓解;7例患者接受手术治疗,5例接受介入治疗。患者维持期治疗以口服泼尼松2.5-15 mg/d,多数患者合用羟基氯喹200 mg/d或沙利度胺50-100 mg/d或甲氨蝶呤10-15 mg/周或硫唑嘌呤50 mg/d,治疗后患者临床症状及血沉（erythrocyte sedimentation rate,ESR）、C-反应蛋白（C-reactive protein,CRP）均得到有效改善。结论：TA女性多发,临床表现异质,以IV型常见,ESR或CRP与疾病活动度并不平行;糖皮质激素联合免疫抑制剂治疗可使多数TA患者病情得到缓解。

Objective：To study the clinical features,radiologic findings,therapeutic strategies and prognosis of the patients with Takayasu arteritis（TA）.Methods：The clinical information and imaging findings of 67 patients with TA were analyzed retrospectively.Results：The ratio of male to female was 1∶4.6.The mean age of first visit was 35（15-74）years with the disease course ranging from 0to 32 years.Fatigue（56.7%）,headache or dizziness（44.8%）,chest discomfort or pain（41.8%）,asymmetric blood pressure（35.8%）were common symptoms followed by extremities chill（11.9%）and numb（13.4%）,and claudication（9.0%）.Angiographic findings showed that typeⅣ（31.3%）was most frequently seen,follwed by typeⅠ（19.4%）.Glucocorticoid was prescribed to 45 patients;in 25 patients,it was combined with cyclophosphamide（CTX）,12 patients with methylaminoperin（MTX）,14 patients with azothioprine（AZA）,1 patient with mycophenolate mofetil（MMF）,7 patients with biological agents;7 patients underwent vascular bypass surgery,and 5 patients underwent endovascular interventional procedures.The levels of erythrocyte sedimentation rate（ESR）and/or C-reactive protein（CRP）were high before treatment in43 patients,and all of them achieved remission.Conclusions：The levels of ESR and CRP are not parallel with the pathological reality.TA patients can achieve remission by treating with glucocorticoid and immunosuppressants.