22. Granulomatosis with Polyangiitis (Wegener's)

  1. Gary S. Hoffman MD, MS2,
  2. Cornelia M. Weyand MD, PhD3,
  3. Carol A. Langford MD, MHS2 and
  4. Jörg J. Goronzy MD, PhD3
  1. Gary S. Hoffman MD, MS2,
  2. Carol A. Langford MD, MHS2 and
  3. Ulrich Specks MD1

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch22

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Hoffman, G. S., Langford, C. A. and Specks, U. (2012) Granulomatosis with Polyangiitis (Wegener's), 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.ch22

Editor Information

  1. 2

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

  2. 3

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

Author Information

  1. 1

    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Pulmonary and Critical Care Medicine, Rochester, MN, USA

  2. 2

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

  • Granulomatosis with polyangiitis (Wegener's);
  • vasculitis;
  • granuloma;
  • ANCA

Summary

Granulomatosis with polyangiitis (Wegener's) (GPA (WG)) is a rare autoimmune disease that affects tissue parenchyma and vessels. Although considered a form of small and medium-sized vessel vasculitis, vascular injury is not a feature of all lesions. While the airways and kidneys have long been considered “signature” sites of disease, any organ can be affected. Antineutrophil cytoplasmic antibodies (ANCA) are likely to have a role in pathogenesis, but disease may occur in its absence. Treatment has vastly changed over the past 40 years. Chronic long-term cyclophosphamide therapy is rarely necessary and discouraged. Morbidity and mortality have been dramatically reduced. Opportunistic infections remain common adverse events and are the leading cause of deaths within the first year of illness. Without a better understanding of etiology, cures will be unlikely, and chronic therapy is usually required.