Petrovic-Rackov Ljiljana, Pejnovic Nada, Jevtic Miodrag, Damjanov Nemanja
Clinic of Rheumatology and Clinical Immunology, Military Medical Academy, Crnotravska 17, 11000 Belgrade, Serbia.
Clin Rheumatol. 2009 Feb;28(2):179-85. doi: 10.1007/s10067-008-1009-7. Epub 2008 Sep 17.
Takayasu's arteritis (TA) is a rare vasculitis that affects the aorta and its primary branches. Heterogeneous clinical manifestations have been described in different geographical areas. We aimed to evaluate demographic, clinical, and angiographic features and the outcome in 16 TA patients who were followed in a single center in Serbia. TA was diagnosed according to the 1990 American College of Rheumatology criteria with median delay of 2 years. The majority of patients (81%) experienced one or more disease relapses and long-term remission was achieved in 94%. Five-year survival rate was 94%. Common systemic manifestations at disease onset included fever (69%), arthralgia (25%), weight loss (19%) and vascular symptoms, arm claudication (100%), headaches (69%), dizziness (56%), and hypertension (37%). All patients had multiple arterial bruits with diminished/absent left arm pulse in 88%. The raised erythrocyte sedimentation rate and C-reactive protein levels were found in 63% and 80%, respectively. Angiography revealed stenotic or occlusive sites of multiple arterial involvement. Stenosis of the left subclavian artery was the most frequent lesion (88%). The angiographic classification was as follows, 50% type I, 19% type IIa, and 31% type V arteritis, suggesting relatively favorable vascular lesions and infrequent serious complications. Glucocorticoids were the main initial therapy for active disease; however, treatment with additional immunosuppressive agents was required in two-thirds of the patients. Seven patients (44%) underwent one or multiple surgical interventions. The main indications for surgery were cerebral hypoperfusion, severe limb claudication, and gangrenous infection. The demographic and angiographic disease patterns in our cohort were similar to American, Italian, Japanese, and Korean cohorts in terms of frequent involvement of the branches of aortic arch and differed from cohorts from other Asian countries in which abdominal aorta and renal artery involvement prevailed.
高安动脉炎（TA）是一种罕见的血管炎，累及主动脉及其主要分支。不同地理区域的临床表现各异。我们旨在评估塞尔维亚单一中心随访的16例TA患者的人口统计学、临床和血管造影特征及预后。TA根据1990年美国风湿病学会标准诊断，中位延迟诊断时间为2年。大多数患者（81%）经历过一次或多次疾病复发，94%实现了长期缓解。五年生存率为94%。疾病发作时常见的全身表现包括发热（69%）、关节痛（25%）、体重减轻（19%）以及血管症状，如上肢间歇性跛行（100%）、头痛（69%）、头晕（56%）和高血压（37%）。所有患者均有多处动脉杂音，88%患者左臂脉搏减弱或消失。分别有63%和80%的患者红细胞沉降率和C反应蛋白水平升高。血管造影显示多处动脉受累的狭窄或闭塞部位。左锁骨下动脉狭窄是最常见的病变（88%）。血管造影分类如下，I型占50%，IIa型占19%，V型动脉炎占31%，提示血管病变相对较轻且严重并发症少见。糖皮质激素是活动性疾病的主要初始治疗药物；然而，三分之二的患者需要加用免疫抑制剂治疗。7例患者（44%）接受了一次或多次手术干预。手术的主要指征是脑灌注不足、严重肢体间歇性跛行和坏疽感染。我们队列中的人口统计学和血管造影疾病模式在主动脉弓分支受累频率方面与美国、意大利、日本和韩国队列相似，与其他亚洲国家的队列不同，后者以腹主动脉和肾动脉受累为主。
