Page 245 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Polyarteritis Nodosa 219

months compared to 17.5% when combined antiviral therapy was offered (P=0.46).
The benefits of adjunctive antiviral therapy appear after prolonged follow-up, since these
drugs lower the risk of cirrhosis or liver failure.

Localized PAN

Localized forms of PAN without poor-prognostic factors can be treated initially without
immunosuppression awaiting refractory or relapsing disease. Isolated appendix or gallbladder
involvement is associated with a favorable prognosis after surgical removal. Cutaneous PAN,
particularly in childhood forms, can be successfully treated with colchicine or dapsone in
combination with topical corticosteroids. Colchicine and dapsone are both effective against
PAN restricted to skeletal muscles.

Conclusion

Polyarteritis nodosa is an exemplary multi-systemic medium-sized, primary systemic
vasculitis of adults and children. Once the most frequent vasculitis, it has become
progressively less common notably in developed countries due to eradication of hepatitis B
virus infection. Effective treatment of polyarteritis nodosa which includes consideration of
corticosteroids and cyclophosphamide depending upon the presence of favorable or
unfavorable prognostic factors, and an antiviral regimen when complicated by hepatitis B
infection, has improved the 5-year survival of patients.

References

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[2] Küssmaul A, Maier R. Ueber eine bischer nicht beschreibene eigenthumliche
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[3] Guillevin L, Mahr A, Callard P, et al. Hepatitis B virus-associated polyarteritis nodosa:
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[4] Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology
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[5] Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill
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[6] Henegar C, Pagnoux C, Puéchal X, et al. A paradigm of diagnostic criteria for
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