Division of Rheumatology, MedStar Georgetown University Hospital, Washington DC, USA; Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD, USA.
Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD, USA.
Semin Arthritis Rheum. 2020 Aug;50(4):576-581. doi: 10.1016/j.semarthrit.2020.04.012. Epub 2020 May 19.
Takayasu's arteritis (TAK) is a clinically heterogenous disease. Patterns of clinical presentation in TAK at diagnosis have not been well described, and a "triphasic pattern" of constitutional symptoms evolving into vascular inflammation and fibrosis has been reported but never systematically evaluated.
Patients with TAK were prospectively recruited from the National Institutes of Health (NIH) and the Vasculitis Clinical Research Consortium (VCRC). Based on clinical presentation at diagnosis, patients were divided into five categories: (1) constitutional symptoms alone, (2) carotidynia, (3) other vascular-associated symptoms, (4) major ischemic event, or (5) asymptomatic. Associated clinical characteristics were evaluated in each category. Preceding symptoms were also assessed to determine the presence of a triphasic disease pattern.
A total of 275 patients with TAK were included (VCRC=208; NIH=67). Similar heterogeneity of clinical presentation was identified in each cohort: constitutional symptoms (8%), carotidynia (13-15%), other vascular symptoms (43-47%), major ischemic event (28-30%), and asymptomatic (2-6%). An increased relative proportion of males was seen in patients who presented with constitutional symptoms or were asymptomatic at diagnosis (p<0.01). Patients who presented with constitutional symptoms and major ischemic events were youngest at diagnosis. Patients in the asymptomatic group were oldest at diagnosis and often were not treated (p<0.01). Relapse was most frequent in patients who presented with carotidynia (p<0.01). A minority of patients (19%) who presented with a major ischemic event reported a triphasic pattern of disease.
There are diverse clinical presentations at diagnosis in TAK. Patients do not necessarily progress sequentially through phases of disease.
Takayasu 动脉炎（TAK）是一种临床表现多样的疾病。TAK 患者在诊断时的临床表现模式尚未得到充分描述，有报道称存在一种“三相模式”，即全身症状逐渐发展为血管炎症和纤维化，但从未得到系统评估。
本前瞻性研究纳入了来自美国国立卫生研究院（NIH）和血管炎临床研究联盟（VCRC）的 TAK 患者。根据诊断时的临床表现，患者被分为以下五类：（1）仅有全身症状；（2）颈动脉炎；（3）其他血管相关症状；（4）主要缺血事件；（5）无症状。在每个类别中评估了相关的临床特征。还评估了前驱症状，以确定是否存在三相疾病模式。
共纳入了 275 例 TAK 患者（VCRC=208；NIH=67）。在每个队列中都发现了相似的临床表现异质性：全身症状（8%）、颈动脉炎（13-15%）、其他血管症状（43-47%）、主要缺血事件（28-30%）和无症状（2-6%）。在以全身症状或无症状为首发表现的患者中，男性的相对比例较高（p<0.01）。以全身症状和主要缺血事件为首发表现的患者诊断时年龄最小。无症状组患者的年龄最大，且往往未接受治疗（p<0.01）。在复发患者中，以颈动脉炎为首发表现的患者最常见（p<0.01）。少数（19%）以主要缺血事件为首发表现的患者报告存在疾病三相模式。
TAK 患者在诊断时有多种临床表现，不一定按疾病阶段依次进展。
