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Common Congenital Heart Lesions - page 111 / 126

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Vasculitic Syndrome: Polyarteritis nodosa (PAN) Clinical Presentation:

  • Generalized inflammatory symptoms:

    • o

      Fever

      • o

        Malaise

      • o

        Musculoskeletal pains

  • Symptoms may also relate to decreased organ blood flow:

      • o

        Hypertension (due to reduced flow into the renal arteries and, thus, renin-angiotensin system activation)

Etiology & some Epidemiology:

  • PAN may be idiopathic (from an obscure or unknown cause).

  • Is also seen with Hepatitis B infection (30% of PAN cases).

  • Prevalence of 6/100,000 – more common in males

Pathology:

  • Many nodules found along course of vessels.

  • Histologic examination of affected areas of arteries shows:

    • o

      PMNs in all three vessel layers (intima, media, adventitia)

      • o

        Intimal proliferation and degeneration

      • o

        Fibrinoid necrosis with occlusion of the lumen

  • Grossly, the vessel wall (including elastic lamina) is disrupted and leads to aneurismal dilatation.

Pathophysiology:

  • Distal to the involved vessel, get ischemia that damages tissues and visceral organs; kidney, heart, liver are

commonly affected.

Diagnostic Imaging/Testing:

  • Biopsy of involved vessel: used to diagnose PAN

  • Blood test: Antineutrophil cytoplasmic antibodies (ANCAs) circulating – suggests necrotizing vasculitis

Treatment:

  • Prednisone and other Immunosuppressive Agents (with treatment, good prognosis: 5 year prognosis of up to 80%)

  • If disease goes untreated, prognosis is poor (5 year = 15%)

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