Role of inflammatory markers in Takayasu arteritis disease monitoring

Abstract

Background: Takayasu arteritis (TA) is an idiopathic large-vessel vasculitis that can result in significant morbidity and mortality secondary to progressive stenosis and occlusion. Monitoring disease progression is crucial to preventing relapse, but is often complicated by the lack of clinical symptoms in the setting of active disease. Although acute phase reactants such as ESR and CRP are generally used as an indicator of inflammation and disease activity, mounting evidence suggests that these markers cannot reliably distinguish active from inactive TA.

Case presentation: We report a 24-year-old Hispanic female with a 5-year history of TA who presented with stroke-like symptoms and evidence of left MCA occlusion on imaging, despite a history of decreasing inflammatory markers. CTA revealed complete occlusion of the left common carotid artery, left subclavian, and left MCA from their origins. It also revealed a striking compensatory circulation supplying the left anterior circulation as well as the left subclavian as a response to progressive stenosis.

Conclusion: Monitoring ESR and CRP levels alone may not be a reliable method to evaluate disease progression in patients with TA, and should be taken in context with both patient's clinical picture and the imaging. We recommend that serial imaging be performed regularly in the setting of active disease to monitor progression and allow for immediate therapy in response to evidence of disease advancement, with a relaxation of the imaging interval once the disease is presumed inactive.

Figures

Figure 1

ESR and CRP values over timespan of treatment.

Figure 2

CTAs three months before CVA. Oblique lateral MPR view CT angiogram of the neck showing a thrombosed left common carotid artery (LCCA) with a high-grade stenosis at the origin of the common carotid, 1 cm in length (A). Transverse CTA just above the origin of the great vessels revealing complete occlusion of the left proximal subclavian artery (LSA) (B), with flow to the distal left subclavian artery provided by the left vertebral artery, indicative of subclavian steal (C).

Figure 3

CTA of the neck immediately following CVA. Anterior (A) and lateral (B) views of a CT angiogram of the neck demonstrating thrombosis of the proximal and mid left common carotid artery (arrows).

Figure 4

Subtracted 3D CT angiogram following CVA. Oblique view showing high-grade stenosis of left MCA with M2 M3 branches opacified by retrograde flow through leptomeningeal collaterals (A). Neither posterior communicating artery (A) nor anterior communicating artery (B) visible upon imaging in oblique and AP views, respectively.

Figure 5

Brain CT imaging post CVA. Brain CT showing completed infarct in left middle cerebral artery territory affecting the left basal ganglia and left insular cortex. Cytotoxic edema present (A). CT perfusion shows increased time to peak (B), reduced cerebral blood volume (C) and reduced cerebral blood flow (D) in the core left MCA territory.

Figure 6

Imaging from 6 months following initial acute CVA. Brain CT showing encephalomalacia related to the previous infarct of the left basal ganglia and left insular cortex (left MCA territory). (A) Transverse slice through neck showing complete occlusion of left common carotid artery (B). Subtracted 3D CT angiogram showing a complete lack of flow in left internal carotid artery as well as a recanalization of the previous area of left MCA thrombosis (C).