Patterns of clinical presentation in Takayasu's arteritis

Abstract

Objective: 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.

Methods: 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.

Results: 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.

Conclusion: There are diverse clinical presentations at diagnosis in TAK. Patients do not necessarily progress sequentially through phases of disease.

Keywords: Epidemiology; Observational cohort; Takayasu's arteritis; Vasculitis.

Figure 1:

The patterns of clinical presentation at diagnosis in Takayasu’s arteritis (TAK) from two independent cohorts [National Institutes of Health (NIH) and Vasculitis Clinical Research Consortium (VCRC)] are detailed. Five categories of clinical presentation at diagnosis were identified in each cohort as follows: constitutional symptoms (8%), carotidynia (13–15%), other vascular symptoms (43–47%), major ischemic event (28–30%), and asymptomatic (2–6%).

Figure 2:

Representative images of patients with various clinical presentations in Takayasu’s arteritis (TAK). A. 21 y/o male with TAK who presented with left-sided carotidynia. Axial STIR image on magnetic resonance (MR) of the carotid arteries demonstrates increased wall thickness and edema in the left common carotid artery (arrow), and fusion to ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET) MR demonstrates associated severe vascular FDG uptake (yellow). Similar to other patients with carotidynia, this patient has relapsing disease and has been treatment refractory. B. 18 year-old male with FDG-PET-computed tomography (CT) demonstrating areas of abnormally low FDG uptake in occipital regions consistent with occipital infarcts (arrow). This patient was diagnosed with TAK when he presented with major ischemic event with resultant blindness. C. 24 year-old female who presented with vascular symptoms (left arm claudication) and was found to have proximal left subclavian artery occlusion (arrow) on MR angiography (MRA). D. Asymptomatic 41 y/o male incidentally found to have descending thoracic aorta aneurysm when he underwent routine chest x-ray. Additional MRA imaging confirmed descending thoracic aorta aneurysm (arrow) and revealed wall thickening in the abdominal aorta, mesenteric artery involvement, and left subclavian artery narrowing.

Figure 3:

The seventy-nine patients who presented with a major ischemic event were evaluated for a ”triphasic” pattern of disease defined as constitutional symptoms evolving into vascular inflammation and ultimately vascular damage. There were 19% patients who had a triphasic disease pattern, 53% patients who had preceding constitutional or vascular symptoms only, and 28% patients who had no preceding symptoms prior to occurrence of major ischemic event.