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Common Congenital Heart Lesions - page 113 / 126

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Vasculitic Syndrome: Giant Cell Arteritis (aka Temporal Arteritis) Clinical Presentation:

  • Symptoms depend on distribution of affected arteries…may include:

    • o

      Prominent headache (from temporal a. involvement)

      • o

        Facial pain

      • o

        Claudication of jaw while chewing (facial a. involvement)

      • o

        50% patients have visual impairment (ophthalmic a. involvement) – irreversible blindness can result

Physical Findings:

  • With Giant Cell Arteritis, may find diminished temporal pulses.

Etiology & some Epidemiology:

  • Associated with Polymyalgia Rheumatica (an inflammatory condition)

  • Uncommon disease (24/100,000).

  • Most often seen in patients > 55 years old.

  • 65% patients are females.

  • Giant Cell Arteritis is usually a self-limiting disease of 1-5 years.

Pathology:

  • Giant Cell Arteritis is a disease of medium to large size arteries: common involvement of cranial vessels, aortic arch and its branches.

  • Histologic examination of affected areas of arteries shows:

    • o

      Lymphocytes infiltrating

      • o

        Intimal fibrosis

      • o

        Focal necrosis with Granulomas containing Multinucleated Giant Cells

Pathophysiology:

  • Distal to the involved vessel, get ischemia that damages tissues.

Diagnostic Imaging/Testing:

  • Erythrocyte Sedimentation Rate: elevated (marker of inflammation)

  • C-reactive Protein: elevated (marker of inflammation)

  • Biopsy of involved vessel (usually a temporal artery): used to diagnose Giant Cell Arteritis

  • Ultrasound (US): can support diagnosis if see a hypoechoic (def: region in US image where echoes are weaker or

fewer than normal) halo around lumen with arterial stenosis and/or occlusion

Treatment:

  • DON’T WAIT FOR BIOPSY RESULTS BEFORE TREATING.

  • Go ahead and give high-dose systemic steroids to treat vasculitis and prevent visual damage

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