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Vascular

Vasculitis

  • Differential Diagnosis
    - SLE
    - Churg Strauss Syndrome
    - Vasculitis from drug abuse
    - Microscopic polyangitis
  • CTA Findings in PAN
    - Multiple aneurysms of varying sizes
    - Smooth narrowing of vessels; stenosis and occlusions of larger vessels
    - Thickening of wall of medium sized vessels
    - Aneurysms may be associated with hemorrhage
    - Bowel wall thickening often with associated strictures or perforation
  • Polyarteritis Nodosa: Facts
    - Most common in 5th-7th decade of life
    - More common in males (2-1)
    - Survival at 5 yrs less than 15% in fulminant disease
    - 50% of patients with abdominal involvement develop acute surgical abdomen with mortality of 12.5%
  • Polyarteritis Nodosa: Clinical Presentation

    Subacute presentation with weight loss, FUO, malaise

    Acute presentation could include;

    • Renal insufficiency or hemorrhage
    • Abdominal pain including ischemia, infarction, or perforation
    • Palpable purpura or ulcerations

     

  • Polyarteritis Nodosa (PAN): Facts
    - Systemic vasculitis causing necrotizing inflammation of small and medium sized vessels, resulting in microaneurysms, occlusions and strictures
    - Kidney is most commonly involved (70-80% of cases)
    - GI tract involved in up to 50% of cases
    - Muscle, skin and CNS are often involved
  • Secondary Vasculitides
    - Sarcoidosis
    - Retroperitoneal fibrosis
    - Inflammatory aneurysm
  • Thromboangitis obliterans: Facts
    - Usually involves young males
    - Strong association with smoking
    - Usually involves the extremities
    - Can lead to superficial thrombophlebitis, raynauds phenomenon, and gangrene
  • Behcets disease: facts
    - Chronic inflammatory vascular disease characterized by recurrent oral and genital aphthous ulcers
    - Arteries of all sizes can be involved and lead to dissection, aneurysm or thrombosis
    - Large vessels involved in up to 25% of cases
  • Small Vessel Vasculitis
    - Wegeners granulomatosis
    - Churg-Strauss syndrome
    - Behcets disease
    - Thromboangitis obliterans
    - Cerebral vasculitis
  • Small Vessel Vasculitis
    - Wegeners granulomatosis
    - Churg-Strauss syndrome
    - both are ANCA associated small vessel vasculities (ANCA-antineutrophilic cytoplasmic antibody)
  • Kawasaki’s Disease: Facts
    - Patients usually younger than age 5 years
    - Involves medium sized vessels
    - Key area of involvement is the coronary arteries where up to 25% of patients may develop aneurysms
  • Medium Sized Vessels
    - Kawasaki’s disease
  • Takayasu’s Arteritis: Facts
    - Usually involves aorta and its main branches
    - Usually in woman
    - More common in Asia
    - May also involve pulmonary arteries and coronary arteries
    - Stenosis often occurs proximately and skip lesions do occur
  • Giant Cell Arteritis: Facts
    - Immune mediated disease characterized by granulomatous infiltration of medium and large arteries
    - Most common presentation Is headache
    - More common in woman usually in the 50’s
    - Vessel wall thickening of 1.5 mm or greater and usually symmetric
  • Large Vessel Vasculitides
    - Giant Cell Arteritis
    - Takayasu’s Arteritis
  • "Large and medium sized vessels are affected by many primary and secondary inflammatory disorders or mimicking conditions, often preventing similar imaging characteristics. Knowledge of distinctive imaging features such as location, length, and degree of vessel wall enhancement as well as the particular clinical settings will help the clinician in appropriately narrowing down the differential diagnosis."

    Imaging of Primary and Secondary Inflammatory Disease Involving Large and Medium Sized Vessels and Their Potential mimics: A Multitechnique Approach
    Spira D et al.
    AJR 2010;194:848-858