Polyarteritis Nodosa Neurologic Manifestations

Hubert de Boysson; Loïc Guillevin

doi:10.1016/j.ncl.2019.01.007

Polyarteritis Nodosa Neurologic Manifestations

Neurol Clin. 2019 May;37(2):345-357. doi: 10.1016/j.ncl.2019.01.007. Epub 2019 Mar 16.

Authors

Hubert de Boysson¹, Loïc Guillevin²

Affiliations

¹ Department of Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France.
² Vasculitides and Scleroderma, Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Université Paris Descartes, 27, rue Fg Saint-Jacques, Paris 75679 Cedex 14, France. Electronic address: loic.guillevin@aphp.fr.

PMID: 30952413
DOI: 10.1016/j.ncl.2019.01.007

Abstract

Polyarteritis nodosa (PAN) is a necrotizing vasculitis affecting medium-sized vessels whose main manifestations are weight loss, fever, peripheral neuropathy, renal, musculoskeletal, gastrointestinal tract and/or cutaneous involvement(s), hypertension and/or cardiac failure. Peripheral neuropathy is one of the most frequent and earliest symptoms, affecting 50% to 75% of PAN patients. Central nervous system involvement affects only 2% to 10% of PAN patients, often late during the disease course. Treatment relies on combining corticosteroids and an immunosuppressant (mainly cyclophosphamide) in patients with poor prognoses. In patients with hepatitis B virus-related PAN, plasma exchanges and antiviral drugs should be combined with corticosteroids.

Keywords: ANCA; Hepatitis B virus; Peripheral neuropathy; Polyarteritis nodosa; Stroke; Vasculitis.

Publication types

Review

MeSH terms

Central Nervous System Diseases / etiology
Humans
Male
Peripheral Nervous System Diseases / etiology
Polyarteritis Nodosa / complications*
Polyarteritis Nodosa / diagnosis
Polyarteritis Nodosa / pathology*