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Common Congenital Heart Lesions - page 112 / 126

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Vasculitic Syndrome: Takayasu’s Arteritis Clinical Presentation:

  • General symptoms:

    • o

      Fever

      • o

        Malaise

  • Focal symptoms related to inflammation of affected vessel:

      • o

        Cerebrovascular ischemia (brachiocephalic or carotid a.)

      • o

        MI (coronary a.)

      • o

        Arm claudication [remember this is a symptom of exertional limb fatigue and pain] (brachiocephalic or subclavian a.)

      • o

        Hypertension (renal a.)

Physical Findings:

  • Takayasu’s Arteritis called “Pulseless disease”…

    • o

      Carotid and limb pulses diminished or absent (85% patients) at time of diagnosis.

  • Possible finding of aortic aneurysm with palpation.

Etiology & some Epidemiology:

  • Idiopathic (from an obscure or unknown cause).

  • Occurs worldwide, but most commonly reported from Asia and Africa

  • Most often seen in women < 40 years old.

Pathology:

  • Takayasu’s arteritis targets the aorta and its major branches.

  • Uncommonly causes aortic aneurysm or aortic dissection.

  • Histologic examination of affected areas of arteries shows:

    • o

      Plasma cells and lymphocytes infiltrating media and adventitia

      • o

        Giant cells

      • o

        Intimal proliferation

      • o

        Disruption of elastic lamina

      • o

        Fibrosis

Pathophysiology:

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

Treatment:

  • Steroid and cytotoxic drugs may reduce vascular inflammation and give symptomatic relief.

  • Surgical bypass of obstructed vessels may be helpful for severe cases.

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