Vasculitis of the nervous system : Current Opinion in Neurology (original) (raw)

Inflammatory diseases and infection

Department of Neurology, New York University School of Medicine, New York, New York 10016, USA

Correspondence to David S. Younger, MD, 715 Park Avenue, Ground Floor, New York, NY 10021, USA Tel: +1 212 535 4314; fax: +1 212 535 6392; e-mail: [email protected]

Abbreviations

ANCA: antineutrophil cytoplasmic antibody

APC: antigen-presenting cell

CNS: central nervous system

CSF: cerebrospinal fluid

GANS: granulomatous angiitis of the nervous system

IVIg: intravenous immunoglobulin

LCV: leukocytoclastic vasculitis

MAC: membrane attack complex

MNM: mononeuritis multiplex

MPA: microscopic polyangiitis

MRI: magnetic resonance imaging

PAN: polyarteritis nodosa

PMN: polymorphonuclear leukocyte

PNS: peripheral nervous system

SLE: systemic lupus erythematosus

SPECT: single photon emission computed tomography

VZV: varicella zoster virus

WG: Wegener granulomatosis

Abstract

Purpose of review

Vasculitis refers to heterogenous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. When unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians.

Recent findings

Remarkable progress has been made in the pathogenesis, diagnosis, and treatment of vasculitis of the central (CNS) and peripheral nervous system (PNS). The classification of vasculitis affecting the nervous system includes (1) Systemic vasculitis disorders (necrotizing arteritis of the polyarteritis type, hypersensitivty vasculitis, systemic granulomatous vasculitis, giant cell arteritis, diverse connective tissue disorders; viral, spirochete, fungal, and retroviral infection; (2) Paraneoplastic disorders; (3) Amphetamine abuse; (4) Granulomatous angiitis of the brain; (5) Isolated peripheral nerve vasculitis, each in the absence of systemic involvement; and (6) diabetes mellitus, associated wtih inflammatoy PNS vasculopathy.

Summary

Vasculitis is diagnosed with assurance after intensive evaluation. Successful treatment follows ascertainment of the specific vasculitic disorder and the underlying cytochemical mechanism of pathogenesis. Clinicians must choose from among the available immunomodulating, immunosuppressive, and targeted immunotherapies, unfortunately without the benefit of prospective clinical trials, tempered by the recognition of all of the possible medication related side effects.