The pathology of large-vessel vasculitides

Dylan V. Miller, J. J. Maleszewski

Research output: Contribution to journalReview articlepeer-review

70 Scopus citations


Vasculitis affecting large elastic arteries, including the aorta and major proximal branches, encompasses various diseases including Takayasu arteritis, giant cell (or temporal) arteritis, and tertiary syphilis, but also may occur as a rare complication of Behçet's disease, rheumatoid arthritis, sarcoidosis, Cogan syndrome, Kawasaki disease, ankylosing spondylitis, systemic lupus erythematosus and Wegener's granulomatosis. Recent reports have also established a link between inflammatory abdominal aortic aneurysm as well as lymphoplasmacytic thoracic aortitis with an overabundance of IgG4-producing plasma cells and the burgeoning constellation of "Hyper-IgG4" syndromes. This review focuses on morphologic aspects of large-vessel vasculitis pathology associated with giant cell arteritis, Takayasu arteritis, idiopathic or isolated aortitis, lymphoplasmacytic thoracic and ascending aortitis, and the inflammatory aneurysm/retroperitoneal fibrosis syndrome.

Original languageEnglish (US)
Pages (from-to)S92-S98
JournalClinical and experimental rheumatology
Issue number1 SUPPL. 64
StatePublished - Jul 21 2011


  • Aortitis
  • Giant cell arteritis
  • IgG4
  • Takayasu

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology


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