Sheikhzadeh A, Tettenborn I, Noohi F, Eftekharzadeh M, Schnabel A
Medical School and Cardiovascular Medical and Research Center, Heart Hospital, Teheran, Iran.
Angiology. 2002 Jan-Feb;53(1):29-40. doi: 10.1177/000331970205300105.
The aim of this study was to evaluate the diagnostic criteria and angiographic classifications of Takayasu arteritis by presenting the clinical, angiographic, and prognostic findings and a prospective follow-up of 78 patients. Occlusive thromboaortopathy or Takayasu arteritis is a large vessel vasculitis. The disease is systemic with an autoimmune and genetic etiology. The complete clinical and angiographic manifestations are reported for 78 cases based on diagnostic criteria of the American College of Rheumatology with a mean 6 +/- 3.2 years follow-up. The mean age was 34.7 and female:male ratio was 3.6:1. According to National Institute of Health criteria, 61.5% of patients were in the acute phase of disease with systemic symptoms such as fever, weight loss, malaise, and elevated C-reactive protein levels. Immunologic markers, such as antinuclear antibody and antineutrophil cytoplasmic antibodies, were negative. The tuberculin test result was positive in 47%. Vascular bruit was present in 89%. Almost all patients had stenoses, occlusions, or aneurysmatic changes of the aorta and its main branches. Hypertension was detected in 58% and left ventricular hypertrophy was initially present in 22 (28%) patients. The angiographic manifestations were classified as type I, cervicobrachial type with 20 cases (25.6%); type II, thoracoabdominal type with 13 cases (16.6%); type III, peripheral type with 10 cases (12.8%); and type IV, generalized type with 35 cases (44.8%). The coronary arteries were involved in 6 cases, pulmonary arteries in 11 initially 5 in follow-up (16 cases), and renal arteries in 28 cases, respectively. A good correlation of the clinical manifestations and the prognosis was observed. During follow-up, five patients suffered from myocardial infarction, six had cerebrovascular accident, seven patients underwent aortic valve replacement, and six patients died (mortality rate, 7.6%). The specificity and sensitivity of diagnostic criteria were 94% and 76%, respectively. In contrast to ours and Nasu's classification in the new classification of Numano, some angiographic types and subtypes of Takayasu arteritis are not present in our patients.
本研究旨在通过呈现78例患者的临床、血管造影及预后结果并进行前瞻性随访，评估大动脉炎的诊断标准及血管造影分类。闭塞性血栓性主动脉病或大动脉炎是一种大血管血管炎。该疾病具有自身免疫和遗传病因，呈全身性。基于美国风湿病学会的诊断标准，报告了78例患者完整的临床和血管造影表现，平均随访时间为6±3.2年。平均年龄为34.7岁，女性与男性比例为3.6:1。根据美国国立卫生研究院标准，61.5%的患者处于疾病急性期，伴有发热、体重减轻、不适及C反应蛋白水平升高等全身症状。抗核抗体和抗中性粒细胞胞浆抗体等免疫标志物均为阴性。结核菌素试验结果47%呈阳性。89%的患者出现血管杂音。几乎所有患者的主动脉及其主要分支均有狭窄、闭塞或动脉瘤样改变。58%的患者检测出高血压，22例（28%）患者最初存在左心室肥厚。血管造影表现分为：I型，颈臂型，20例（25.6%）；II型，胸腹型，13例（16.6%）；III型，外周型，10例（12.8%）；IV型，全身型，35例（44.8%）。冠状动脉受累6例，肺动脉最初受累11例，随访期间5例（共16例），肾动脉受累28例。观察到临床表现与预后具有良好的相关性。随访期间，5例患者发生心肌梗死，6例发生脑血管意外，7例患者接受主动脉瓣置换，6例患者死亡（死亡率7.6%）。诊断标准的特异性和敏感性分别为94%和76%。与我们以及Numano新分类中Nasu的分类不同，我们的患者中不存在某些大动脉炎的血管造影类型和亚型。
