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Vasculitides

B. Polyarteritis Nodosa
  • Keith T. Rott

Abstract

  • 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.

Keywords

Giant Cell Arteritis Systemic Vasculitis Hairy Cell Leukemia Polyarteritis Nodosa Hemorrhagic Cystitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    Kussmaul A, Maier R. Ueber eine bisher nicht beschriebene eigenthumliche arterienerkrankung (periarteritis nodosa), die mit morbus brightii und rapid fortschreitender allgemeiner muskellahmung einhergeht. Dtsch Arch Klin Med 1866;1:484–518.Google Scholar
  2. 2.
    Matteson EL. Polyarteritis nodosa: commemorative translation of the 130-year anniversary of the original article by Adolf Kussmaul and Rudolf Maier. Rochester, MN: Mayo Foundation; 1996.Google Scholar
  3. 3.
    Jennette J, Falk R, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187–192.PubMedCrossRefGoogle Scholar
  4. 4.
    Stone JH. Polyarteritis nodosa. JAMA 2002;288:1632–1639.PubMedCrossRefGoogle Scholar
  5. 5.
    Lightfoot RW, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classifi cation of polyarteritis nodosa. Arthritis Rheum 1990;33:1088–1093.PubMedCrossRefGoogle Scholar
  6. 6.
    Levine SM, Hellman DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients. Am J Med 2002;112:386–391.PubMedCrossRefGoogle Scholar
  7. 7.
    Tervaert JWC, Kallenberg C. Neurologic manifestations of systemic vasculitides. Rheum Dis Clin North Am 1993;19:913–940.PubMedGoogle Scholar
  8. 8.
    Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am 1995;21:1097–1113.PubMedGoogle Scholar
  9. 9.
    Conn DL. Polyarteritis. Rheum Dis Clin North Am 1990;16:341–362.PubMedGoogle Scholar
  10. 10.
    Hunder GH, Arend WP, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Arthritis Rheum 1990;33:1065–1067.PubMedCrossRefGoogle Scholar
  11. 11.
    Gayraud M, Guillevin L, Le Toumelin P, et al. Long term follow up of polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Analysis of four prospective trials including 278 patients. Arthritis Rheum 2001;44:666–675.PubMedCrossRefGoogle Scholar
  12. 12.
    Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 1996;75:17–28.CrossRefGoogle Scholar
  13. 13.
    De Groot K, Adu D, Savage COS. The value of pulse cyclophosphamide in ANCA-associated vasculitis: metaanalysis and critical review. Nephrol Dial Transplant 2001;16:2018–2027.PubMedCrossRefGoogle Scholar

Copyright information

© 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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