1st Department of Internal Medicine, Agios Dimitrios General Hospital, Thessaloniki, Greece.
Clin Exp Rheumatol. 2009 Jan-Feb;27(1 Suppl 52):S33-9.
Takayasu arteritis (TA) is an uncommon disease with clinical heterogeneity across different ethnic groups. We aimed to evaluate the epidemiological, clinical, and immuno-genetic features of TA in Greece.
Demographic, clinical, laboratory, angiographic, and therapeutic data of 42 patients from 4 large referral centers were retrieved. Serology and Human Lymphocyte Antigen (HLA) typing was performed in 22 patients.
We studied 37 women and 5 men with a median age of 31 years at disease onset. Median delay in diagnosis was 24 months and median follow-up was 47 months (range 0-178). Constitutional or musculoskeletal symptoms were present in 86%, especially early in the disease course. Vascular findings were universal with reduced or absent pulse being the most common manifestation (98%). Hypertension was frequent (78%). Extensive disease prevailed and stenotic lesions were more common than aneurysms (95% vs. 40%). Erythrocyte sedimentation rate and C-reactive protein showed modest correlation with disease activity. HLA-B52 was expressed by 37% of the patients vs. 2.4% of the controls (p<0.001). Glucocorticoids and cytotoxic agents were used in most patients with remission rates of 83%. A total of 42 surgical procedures were performed with success rates of 87%.
TA in Greece clinically and epidemiologically resembles the pattern of disease in Japan and the Western hemisphere. There is considerable delay in diagnosis, which may partially reflect failure to recognize a rare disease. New surrogate markers are needed to assess disease activity. Glucocorticoids are the cornerstone of treatment and cytotoxic drugs are frequently used as steroid sparing agents.
Takayasu 动脉炎（TA）是一种少见疾病，在不同种族之间具有临床异质性。我们旨在评估希腊 TA 的流行病学、临床和免疫遗传特征。
从 4 个大型转诊中心检索了 42 名患者的人口统计学、临床、实验室、血管造影和治疗数据。对 22 名患者进行了血清学和人类淋巴细胞抗原（HLA）分型。
我们研究了 37 名女性和 5 名男性，发病时的中位年龄为 31 岁。中位诊断延迟时间为 24 个月，中位随访时间为 47 个月（范围 0-178）。86%的患者存在全身或肌肉骨骼症状，尤其是在疾病早期。血管表现普遍存在，无脉或脉搏减弱是最常见的表现（98%）。高血压很常见（78%）。广泛的疾病占主导地位，狭窄性病变比动脉瘤更常见（95%比 40%）。红细胞沉降率和 C 反应蛋白与疾病活动度有一定的相关性。HLA-B52 在 37%的患者中表达，而在对照组中为 2.4%（p<0.001）。大多数患者使用糖皮质激素和细胞毒性药物治疗，缓解率为 83%。共进行了 42 例手术，成功率为 87%。
希腊的 TA 在临床和流行病学方面与日本和西半球的疾病模式相似。诊断存在相当大的延迟，这可能部分反映了对罕见疾病的认识不足。需要新的替代标志物来评估疾病活动度。糖皮质激素是治疗的基石，细胞毒性药物经常被用作类固醇保留剂。
