24. Giant Cell Arteritis

  1. Gary S. Hoffman MD, MS1,
  2. Cornelia M. Weyand MD, PhD2,
  3. Carol A. Langford MD, MHS1 and
  4. Jörg J. Goronzy MD, PhD2
  1. Cornelia M. Weyand MD, PhD and
  2. Jörg J. Goronzy MD, PhD

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch24

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Weyand, C. M. and Goronzy, J. J. (2012) Giant Cell Arteritis, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch24

Editor Information

  1. 1

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 2

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244

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Keywords:

  • Giant cell arteritis;
  • temporal arteritis;
  • vasculitis;
  • dendritic cell;
  • T cell

Summary

Giant cell arteritis (GCA), a primary angiitis of large and medium-sized arteries, occurs almost exclusively during the sixth to eighth decades of life. Pathogenic events are initiated by organ-specific dendritic cells (DC) close to the vasa vasorum network of three-layered arteries. Such DC participate in immunosurveillance and recognize pathogen-associated and danger-associated molecular patterns. Early inflammation is mediated by Th17 cells; chronic vasculitis relies on IFN-γ-producing Th1 cells suggesting two separable pathogenic pathways. Temporal artery biopsy remains the diagnostic approach of choice. Imaging of the vascular lumen and vessel wall by magnetic resonance imaging or computed tomography are instrumental to capture large vessel involvement. GCA is treated with corticosteroids; convincing data that other immunosuppressants have significant steroid-sparing action or can treat steroid-resistant disease is currently lacking. Whether GCA enters remission is unknown and whether continuous immunosuppression prevents sequel of smoldering vasculitis is not established. Avoiding corticosteroid overuse is critical in patient care. Skillful management requires accurate distinction between aggressive inflammation (high dose therapy necessary), stable disease (watchful monitoring) and progressive vascular damage (low dose, long-term treatment may be warranted).