B. Polyarteritis Nodosa
  • Keith T. Rott


  • Polyarteritis nodosa (PAN) primarily affects mediumsized arteries that supply the skin, gut, nerve, and kidney, but may involve multiple organs.

  • Microaneurysms of arteries to or within the kidneys, liver, or gastrointestinal tract are highly characteristic of PAN.

  • Polyarteritis nodosa is not associated with antineutrophil cytoplasmic antibodies (ANCA) directed against either proteinase-3 or myeloperoxidase.

  • Mononeuritis multiplex, an asymmetric sensory and motor neuropathy due to ischemia and infarction of peripheral nerves, occurs frequently in PAN.

  • In mononeuritis multiplex, nerve conduction studies of peripheral nerves reveal a distal, asymmetric, axonal neuropathy involving both motor and sensory nerves.

  • Polyarteritis nodosa is characterized pathologically by patchy, transmural inflammation in medium- and small-sized muscular arteries, sparing large arteries, capillaries, and the venous system. The inflammation leads to fibrinoid necrosis, but is not associated with granulomatous features.

  • High-dose glucocorticoids are the mainstay of therapy in PAN. In cases that are rapidly progressive or life- or organ-threatening, however, cyclophosphamide is added to glucocorticoid treatment.

  • Most cases of idiopathic PAN do not recur once a sound remission has been achieved with 6 to 12 months of therapy.

  • ■ A minority of PAN cases (now >10%) are associated with acute hepatitis B infection. Cases associated with hepatitis B are treated with regimens emphasizing antiviral therapy and only short courses of immunosuppression and plasmapheresis.


Giant Cell Arteritis Systemic Vasculitis Hairy Cell Leukemia Polyarteritis Nodosa Hemorrhagic Cystitis 
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© Springer Science+Business Media, LLC. 2008

Authors and Affiliations

  • Keith T. Rott
    • 1
  1. 1.Department of Medicine, Division of RheumatologyEmory University School of MedicineAtlantaUSA

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