Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge

L Watson,I Peart,C Landes,N Barnes,P Brogan,G Cleary

doi:10.1155/2014/603171

Abstract

Takayasu Arteritis (TA) is a rare, debilitating large vessel vasculitis occurring in patients of all ages, including infants, but the disease most commonly presents in the third decade. Diagnosis is often delayed and consequently TA is associated with significant morbidity and mortality. Accurate methods of monitoring disease activity or damage are lacking and currently rely on a combination of clinical features, blood inflammatory markers, and imaging modalities. In this report we describe a case of a 14-year-old boy with childhood-onset TA who, despite extensive negative investigations, did indeed have on-going active large vessel vasculitis with fatal outcome. Postmortem analysis demonstrated more extensive and active disease than originally identified. This report illustrates and discusses the limitations of current modalities for the detection and monitoring of disease activity and damage in large vessel vasculitis. Clinicians must be aware of these limitations and challenges if we are to strive for better outcomes in TA.

Highlights

Takayasu Arteritis (TA) is a large vessel vasculitis of unknown aetiology that affects the aorta and its branches
Diagnosing TA relies on clinical presentation, characteristic structural arterial abnormalities, and evidence of inflammatory vasculopathy on imaging or histology
A classification of childhood TA exists (Table 1); the major clinical challenge when considering the disease is distinguishing this inflammatory large vessel vasculitis from other noninflammatory vasculopathies, since the differential diagnosis is broad (Table 2). This issue is difficult when the disease is in the late stenotic phase, where acute vasculitic disease activity may be minimal or absent

Summary

Introduction

Takayasu Arteritis (TA) is a large vessel vasculitis of unknown aetiology that affects the aorta and its branches. It is important to identify active disease since this requires immunosuppressive treatment, but it is unclear whether immunosuppression is effective in the late stage of the disease when a risk-benefit balance must be made [5]. Revascularisation procedures, such as angioplasty and/or stenting, or more invasive surgical interventions such as aortic bypass grafting are commonly required to provide relief from symptomatic ischaemia but are associated with significant life threatening risks and morbidity and are not completely effective in all cases [6, 7]. We present a case that highlights the difficulties clinicians face in relation to the diagnosis and monitoring of large vessel vasculitis

Case Presentation

Discussion