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Issue: February 1, 2007
Case: Vasculitis
Taken from
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Question:

Polyarteritis nodosa (PAN) is histologically characterized by medial fibrinoid necrosis of the small and medium sized arteries in virtually any organ. All of the following signs and symptoms are consistent with PAN EXCEPT:

  1. acute onset of fever, chills, arthralgias and myalgias
  2. mononeuritis multiplex
  3. pneumonitis, alveolar infiltrates, pulmonary hemorrhage and/or hemothorax
  4. pericarditis, myocarditis, or myocardial infarction
  5. proteinuria, hematuria, hypertension, and renal failure
  6. retinal vasculitis on funduscopic examination
  7. painful violaceous plaques with ulceration and livedo reticularis
  8. hepatitis with transaminitis; intestinal and gallbladder inflammation

Answer:
C

Remediation:
Pulmonary involvement is unusual or rare (though bronchial vessels may be involved) in classic PAN.

"Microscopic polyangiitis" is a similar disorder affecting the small vessels. It may overlap with PAN, and commonly includes glomerulonephritis and pulmonary capillaritis.

Mortality is high without treatment as patients are at risk for intestinal perforation, renal failure, and heart failure. The renal disease of classic PAN is predominantly due to arteritis and subsequent ischemic renal failure, whereas microscopic polyangiitis is commonly associated with glomerulonephritis.

Acute flares of PAN are treated with prednisone, 2 mg/kg/day (80 mg maximum) or IV methylprednisolone, possibly combined with cyclophosphamide PO or IV for 3 days.

About the Image(s):
Image 1:
Polyarteritis Nodosa on the Arm with Livedo Reticularis
PAN is characterized by a small and medium vessel vasculitis in the deep dermis. Resultant necrotic plaques, subcutaneous nodules, and livedo reticularis can also be seen.


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