Department of Rheumatology at Yeditepe University, Faculty of Medicine, Istanbul, Turkey.
Clin Exp Rheumatol. 2009 Jan-Feb;27(1 Suppl 52):S59-64.
Takayasu's arteritis (TA) is a chronic, inflammatory vasculitis affecting the aorta and its major branches. Although it is more prevalent in Far-East Asia, the distribution of the disease is worldwide with different vascular involvement patterns and clinical manifestations. The objective of this study was to evaluate the demographic, clinical, angiographic and prognostic features of TA patients in Turkey.
Clinical and angiographic findings of 248 TA patients (228 female, 27 male) followed at 15 Rheumatology Centers were prospectively evaluated according to a predefined protocol.
The mean age was 40.1 years (30.2 years at the clinical onset). Clinical manifestations included constitutional symptoms in 66%, absent or diminished pulses in 88%, bruits in 77%, extremity pain in 69%, claudication in 48%, hypertension in 43% and cerebrovascular accidents (CVA) in 18% of the patients. Renal artery stenosis, aortic regurgitation and pulmonary hypertension were present in 26%, 33% and 12%, respectively. According to the new angiographic classification, type V (50.8%) and Type I (32%) were the most frequent types of involvement. Corticosteroids were the main treatment in 93% of the patients alone (9%) or in combination with immunosuppressive agents (84%). Most frequently preferred immunosuppressive agents were methotrexate (63%), azathioprine (22%) and cyclophosphamide (13%). Remission was observed at least once in 94% of the patients and sustained remission in 71% during follow-up.
The demographical, clinical and angiographic findings of TA patients in our series were similar to those reported from Japan, Brazil and Colombia. Combination therapies with immunosuppressive agents were the preferred choice of treatment in Turkey.
Takayasu 动脉炎(TA)是一种慢性炎症性血管炎,影响主动脉及其主要分支。尽管它在远东地区更为普遍,但该病的分布是全球性的,具有不同的血管受累模式和临床表现。本研究的目的是评估土耳其 TA 患者的人口统计学、临床、血管造影和预后特征。
根据预设方案,前瞻性评估了 15 个风湿病中心随访的 248 例 TA 患者(228 例女性,27 例男性)的临床和血管造影发现。
平均年龄为 40.1 岁(临床发病时为 30.2 岁)。临床表现包括 66%的患者有全身症状,88%的患者无或减弱脉搏,77%的患者有杂音,69%的患者肢体疼痛,48%的患者跛行,43%的患者高血压,18%的患者发生脑血管意外(CVA)。肾动脉狭窄、主动脉瓣反流和肺动脉高压分别见于 26%、33%和 12%的患者。根据新的血管造影分类,V 型(50.8%)和 I 型(32%)是最常见的受累类型。93%的患者单独(9%)或联合免疫抑制剂(84%)接受皮质类固醇治疗。最常选用的免疫抑制剂为甲氨蝶呤(63%)、硫唑嘌呤(22%)和环磷酰胺(13%)。94%的患者至少有一次缓解,71%的患者在随访期间维持缓解。
我们系列中的 TA 患者的人口统计学、临床和血管造影结果与日本、巴西和哥伦比亚的报告相似。联合免疫抑制剂的联合治疗是土耳其的首选治疗方法。