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Case Reports in Rheumatology
Volume 2014, Article ID 603171, 6 pages
http://dx.doi.org/10.1155/2014/603171
Case Report

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

1Department of Paediatric Nephrology, Institute of Child Health, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK
2Department of Paediatric Rheumatology, Great Ormond Street NHS Hospital, London WC1N 3JH, UK
3Department of Paediatric Cardiology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK
4Department of Paediatric Radiology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK
5Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK

Received 23 August 2013; Accepted 28 October 2013; Published 5 January 2014

Academic Editors: S. Ikuyama, A. Mak, C. Saadeh, and G. J. Tsay

Copyright © 2014 L. Watson et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.