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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Wiley
2014-01-01
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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. |
format | Article |
id | doaj-art-b6b65103309c49ef84b4010e0a4c5cca |
institution | Kabale University |
issn | 2090-6889 2090-6897 |
language | English |
publishDate | 2014-01-01 |
publisher | Wiley |
record_format | Article |
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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