Page 223 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

In: The Vasculitides, Volume 1 ISBN: 978-1-63463-110-5
Editors: David S. Younger © 2015 Nova Science Publishers, Inc.

Chapter 11

Polyarteritis Nodosa

Loic Guillevin, M.D.?

Professor of Medicine, Department of Internal Medicine,
National Referral Center for Rare Autoimmune and Systemic Diseases,

Hôpital Cochin, Assistance Publique–Hôpitaux de Paris,
University of Paris 5-René-Descartes, Paris, France

Abstract

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, one of its most frequent causes. The evolution of clinical
manifestations commences with the acute phase of neutrophilic and variable lymphocytic
and eosinophilic inflammation of arterial vessel walls with medial necrosis, followed
aneurysm formation, and later healing of lesions that entrains fibrotic endarteritis and
vascular occlusions. The coexistence of necrotizing vasculitis, healed lesions and normal
arteries in different tissues or portions of the same tissue is a pathognomonic feature of
the disease. 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 agent if complicated by hepatitis B
infection, have improved the 5-year survival of patients. This chapter considers aspects of
the classification criteria, epidemiology, main clinicopathologic features,
etiopathogenesis, treatment and outcome of PAN.

Keywords: Polyarteritis nodosa, vasculitis, CNS, PNS, Systemic vasculitis

? Correspondence: Loic Guillevin, Department of Internal Medicine, National Referral Center for Rare
Autoimmune and Systemic Diseases, INSERM U1060, Hôpital Cochin, Assistance Publique–Hôpitaux de
Paris, University of Paris 5-René-Descartes, 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14,
France. Email: [email protected]

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