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

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 me...

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Main Authors: L. Watson, P. Brogan, I. Peart, C. Landes, N. Barnes, G. Cleary
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2014/603171
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author L. Watson
P. Brogan
I. Peart
C. Landes
N. Barnes
G. Cleary
author_facet L. Watson
P. Brogan
I. Peart
C. Landes
N. Barnes
G. Cleary
author_sort L. Watson
collection DOAJ
description 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.
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series Case Reports in Rheumatology
spelling doaj-art-b6b65103309c49ef84b4010e0a4c5cca2025-02-03T05:51:43ZengWileyCase Reports in Rheumatology2090-68892090-68972014-01-01201410.1155/2014/603171603171Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the ChallengeL. Watson0P. Brogan1I. Peart2C. Landes3N. Barnes4G. Cleary5Department of Paediatric Nephrology, Institute of Child Health, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UKDepartment of Paediatric Rheumatology, Great Ormond Street NHS Hospital, London WC1N 3JH, UKDepartment of Paediatric Cardiology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UKDepartment of Paediatric Radiology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UKDepartment of Paediatric Radiology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UKDepartment of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UKTakayasu 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.http://dx.doi.org/10.1155/2014/603171
spellingShingle L. Watson
P. Brogan
I. Peart
C. Landes
N. Barnes
G. Cleary
Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge
Case Reports in Rheumatology
title Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge
title_full Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge
title_fullStr Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge
title_full_unstemmed Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge
title_short Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge
title_sort diagnosis and assessment of disease activity in takayasu arteritis a childhood case illustrating the challenge
url http://dx.doi.org/10.1155/2014/603171
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