Large vessel vasculitides

Large vessel vasculitides

GCA and TA are the most common phenotypes of primary large-vessel vasculitis. New classification criteria including new imaging techniques are under development for these two large-vessel vasculitides. Other rheumatic diseases associated with aortitis include the seronegative spondyloarthropathies, relapsing polychondritis, Behçet’s disease, Cogan’s syndrome, and sarcoidosis. Infectious aortitis may be caused by several organisms. In patients without systemic symptoms who present with a thoracic aortic aneurysm, several noninflammatory genetic conditions must be ruled out.

Pearl: the differential can be narrowed by the vascular territory predominantly involved. GCA and TA most frequently involve the ascending aorta and left subclavian artery.

  • a) Ascending aortic involvement.
    • • Involving the arch, subclavians, and carotids.
    • • Inflammatory: GCA, TA, Behçet’s.
    • • Noninflammatory (genetic): familial thoracic aortic aneurysms and dissection, Marfans, Ehlers–Danlos type IV, Loeys–Dietz.
  • b) Isolated descending aortic involvement.
    • • TA (India, Pakistan).
    • • Inflammatory abdominal aortic aneurysm.
  • c) Isolated pulmonary artery involvement.
    • • Hughes–Stovin syndrome (Behçet’s).
  • d) Periaortic involvement.
    • • IgG4-related disease (IgG4-RD).
    • • Lymphoma.
    • • Erdheim–Chester.

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