Granulomatosis with Polyangiitis (Wegener's)

Gary S. Hoffman, Carol A. Langford, Ulrich Specks

Research output: Chapter in Book/Report/Conference proceedingChapter

4 Scopus citations

Abstract

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.

Original languageEnglish (US)
Title of host publicationInflammatory Diseases of Blood Vessels
Subtitle of host publicationSecond Edition
PublisherWiley-Blackwell
Pages238-251
Number of pages14
ISBN (Print)9781444338225
DOIs
StatePublished - May 3 2012

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Keywords

  • ANCA
  • Granuloma
  • Granulomatosis with polyangiitis (Wegener's)
  • Vasculitis

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Hoffman, G. S., Langford, C. A., & Specks, U. (2012). Granulomatosis with Polyangiitis (Wegener's). In Inflammatory Diseases of Blood Vessels: Second Edition (pp. 238-251). Wiley-Blackwell. https://doi.org/10.1002/9781118355244.ch22