目的:评估Takayasu动脉炎(TAK)的损伤和与损伤相关的临床特征。
方法:在多中心登记的TAK患者,prospective,观察性研究每6个月使用血管炎损伤指数(VDI)和大血管血管炎损伤指数(LVVID)进行标准化损伤评估.
结果:该研究包括236例TAK患者:92%为女性,81%白种人;中位数(第25位,第75百分位数)疾病持续时间=2.6(0.12,6.9)年。84%的人进行了随访:中位数(第25位,第75年)持续时间4.1(1.9,7.5)年。VDI上有89%的损坏项目,LVVID为87%,在外周血管(76%VDI,74%LVVID),心脏(40%VDI,45%LVVID)系统。随访期间,42%的患者有新的损伤;包括主要血管狭窄/动脉闭塞(8%),肢体跛行(6%),高血压(7%),主动脉瘤(4%),和搭桥手术(4%)。疾病特异性损害占>90%的新项目。年纪大了,复发,随访时间较长与新的损伤项目相关;没有新损伤的患者使用甲氨蝶呤的比例较高(p<0.05).在入选后180天内诊断为TAK的48例患者中,新的损伤发生在31%的VDI和52%的LVVID。在整个队列中,复发史与新的损伤相关,而在最近诊断的患者中,诊断时年龄较大与新的损伤相关.
结论:即使最近诊断,>80%的TAK患者也存在损伤,>40%的患者出现新的损伤,主要是疾病特异性损害。应优先考虑更好地控制疾病活动和防止损害的TAK疗法。
OBJECTIVE: To evaluate damage and clinical characteristics associated with damage in Takayasu\'s arteritis (TAK).
METHODS: Patients with TAK enrolled in a multicentre, prospective, observational study underwent standardized damage assessment every 6 months using the Vasculitis Damage Index (VDI) and the Large-Vessel Vasculitis Index of Damage (LVVID).
RESULTS: The study included 236 patients with TAK: 92% female, 81% Caucasian; median (25th, 75th percentile) disease duration = 2.6 (0.12, 6.9) years. Eighty-four percent had follow-up: median (25th, 75th) duration 4.1 (1.9, 7.5) years.Items of damage were present in 89% on VDI, 87% on LVVID, in the peripheral vascular (76% VDI, 74% LVVID), cardiac (40% VDI, 45% LVVID) systems. During follow-up, 42% patients had new damage;, including major vessel stenosis/arterial occlusion (8%), limb claudication (6%), hypertension (7%), aortic aneurysm (4%), and bypass surgery (4%). Disease-specific damage accounted for >90% new items. Older age, relapse, and longer duration of follow-up were associated with new damage items; a higher proportion of patients without new damage were on methotrexate (p< 0.05). Among 48 patients diagnosed with TAK within 180 days of enrolment, new damage occurred in 31% on VDI and 52% on LVVID. History of relapse was associated with new damage in the entire cohort while in patients with a recent diagnosis, older age at diagnosis was associated with new damage.
CONCLUSIONS: Damage is present in > 80% of patients with TAK even with recent diagnosis and >40% of patients accrue new, mainly disease-specific damage. Therapies for TAK that better control disease activity and prevent damage should be prioritized.