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Takayasu arteritis—advances in diagnosis and management

Abstract

Takayasu arteritis, a rare granulomatous vasculitis affecting young people, is associated with considerable morbidity and premature mortality. In most patients the diagnosis is delayed until after the development of substantial arterial injury. Studies of noninvasive imaging techniques suggest that these approaches might facilitate earlier diagnosis and have a role in monitoring disease progress; however, they remain limited in their ability to accurately quantify inflammatory disease activity in the arterial wall. A lack of controlled clinical trial data complicates the choice of therapy for Takayasu arteritis, and clinical indices for monitoring disease activity are currently suboptimal. Increasing knowledge of the pathogenesis of the large vessel vasculitides might eventually lead to novel targeted therapies. Preliminary data from open-label trials of anti-tumor necrosis factor therapy are encouraging, but there is an urgent need for controlled clinical trials to establish optimum therapeutic approaches for this disease. These trials should include a prospective assessment of the use of noninvasive imaging modalities in the diagnosis and subsequent management of Takayasu arteritis.

Key Points

  • The rarity of Takayasu arteritis and its heterogeneous clinical presentation typically results in delayed diagnosis and extensive arterial injury

  • Current outcomes for patients are relatively poor, with substantial morbidity and considerable mortality

  • Treatment decisions are complicated by the lack of available clinical trial data

  • 18FDG-CT-PET, MRA, CTA and US imaging might allow diagnosis of pre-stenotic disease and noninvasive monitoring of arterial injury; however, they remain limited in their ability to monitor disease activity

  • Controlled clinical trials are required to establish optimum therapeutic approaches, to demonstrate a beneficial effect on vascular outcome and to determine the utility of noninvasive imaging techniques

  • Improved understanding of disease pathogenesis and early trials of anti-tumor necrosis factor therapy offers the prospect of a targeted biological approach to treatment in the future

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Figure 1: Imaging in Takayasu arteritis.
Figure 2: MRA in Takayasu arteritis.
Figure 3: Longitudinal ultrasound images of the common carotid artery.
Figure 4: PET in Takayasu arteritis.
Figure 5: Surgical intervention for severe distal stenosis of the abdominal aorta.
Figure 6: Novel therapeutic targets.

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Acknowledgements

I acknowledge support from the National Institute for Health Research Biomedical Research Centre funding scheme. I would to thank my colleagues Adil Al-Nahhas, Syed Babar and Dorian Haskard (Imperial College Healthcare NHS Trust, London), Raad Mohiaddin, Niall Keenan and Dudley Pennell (Royal Brompton Hospital, London), Paolo Camici, Ornella Rimoldi and Oliver Gaemperli (Medical Research Council Clinical Sciences Center, Imperial College, London) and Jackie Andrews (Leeds Teaching Hospitals NHS Trust) for their expertise and support, and our patients for their willingness to be involved in research. Désirée Lie, University of California, Irvine, CA is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Mason, J. Takayasu arteritis—advances in diagnosis and management. Nat Rev Rheumatol 6, 406–415 (2010). https://doi.org/10.1038/nrrheum.2010.82

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