Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients
Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients
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DOI：
10.1002/art.22404
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发表时间：
2007-02-01
影响因子：
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通讯作者：
Hoffman, Gary S.
中科院分区：
文献类型：
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作者：
Maksimowicz-McKinnon, Kathleen;Clark, Tiffany M.;Hoffman, Gary S.
Objective.描述来自美国的一个队列中的大动脉炎（TA）的临床、实验室和放射学表现，评价对干预的反应、缓解和复发率以及疾病进展，并将这些观察结果与来自美国、日本、印度、意大利和墨西哥的其他队列的观察结果进行比较。使用统一的数据库，包括临床，实验室和影像学数据的75例患者进行了回顾性研究。至少每年进行一次血管成像检查以监测疾病进展。发病时常见的表现包括脉搏消失或不对称（57%），肢体血压差异（53%）和杂音（53%）。11%的患者在疾病诊断前无症状。最初的血管造影研究显示79%的患者存在主动脉异常，锁骨下动脉（65%）和颈动脉（43%）频繁受累，93%的纵向随访患者获得了任何持续时间的疾病缓解，但只有28%的患者在泼尼松逐渐减少至< 10 mg/d后至少持续缓解6个月。血管成形术和血管外科手术最初都是成功的，但78%的血管成形术和36%的旁路/重建手术发生了复发性狭窄。超过三分之二的患者难以进行日常活动，约四分之一的患者无法工作。我们的队列在女性受试者和疾病表现方面与美国国立卫生研究院、意大利、日本和墨西哥队列相似，但与印度队列不同的是，后者的男性受试者频率更高，腹主动脉和肾动脉受累与高血压。虽然糖皮质激素治疗后TA症状通常会改善，但减少剂量通常会复发。恢复血管通畅的尝试通常最初是成功的，但再狭窄经常发生。在美国，大多数TA患者发生慢性发病和残疾。
Objective. To describe the clinical, laboratory, and radiographic manifestations of Takayasu arteritis (TA) in a cohort from the US, evaluate the response to interventions, remission and relapse rates, and disease progression, and compare these observations with those from other cohorts in the US, Japan, India, Italy, and Mexico.Methods. Seventy-five patients were retrospectively studied using a uniform database that included clinical, laboratory, and imaging data. Vascular imaging studies were performed at least yearly to monitor disease progression.Results. Common manifestations at disease onset included loss or asymmetry of pulses (57%), limb blood pressure discrepancy (53%), and bruits (53%). Eleven percent of patients were asymptomatic prior to disease diagnosis. Initial angiographic studies showed aortic abnormalities in 79% of patients and frequent involvement of the subclavian (65%) and carotid (43%) arteries.Ninety-three percent of longitudinally followed patients attained disease remission of any duration, but only 28% sustained remission of at least 6 months' duration after prednisone was tapered to < 10 mg daily. Both angioplasty and vascular surgery were initially successful, but recurrent stenosis occurred in 78% of angioplasty and 36% of bypass/reconstruction procedures. More than two-thirds of patients had difficulty performing routine daily activities and approximately one-fourth of all patients were unable to work.Our cohort was similar to the National Institutes of Health, Italian, Japanese, and Mexican cohorts in terms of the predominance of female subjects and disease manifestations, but differed from the Indian cohort in that the latter group had a higher frequency of male subjects, abdominal aorta and renal artery involvement, and hypertension.Conclusion. Although improvement of symptoms in TA usually follows glucocorticoid therapy, relapses usually occur with dosage reduction. Attempts to restore vascular patency are often initially successful, but restenosis occurs frequently. Chronic morbidity and disability occur in most patients with TA in the US.