Central Nervous System Vasculitis

Ludovico D'Incerti, Francesco Deleo, Gabriele Di Comite

Research output: Chapter in Book/Report/Conference proceedingChapter


This chapter provides insight into the relevant clinical, pathogenetic, diagnostic, and therapeutic aspects of the central nervous system (CNS) involvement in primary and secondary vasculitides. Vasculitides represent a group of highly heterogeneous disorders characterized by inflammation in the wall of blood vessels. Vasculitis, an immune-mediated mechanism, can occur as a primary process or can be secondary to an underlying condition, including infections, malignancies, connective tissue diseases, and drug use. The classification of vasculitides has been extremely challenging over the decades and several attempts have been made to classify them according to etiology, the clinical picture, or the underlying immunopathologic mechanism. In this regard, a major advance was achieved in 1990 when the American College of Rheumatology (ACR) published a set of classification criteria, grouping primary vasculitides according to the size of affected vessels. They identified three groups: small-vessel, medium-vessel, and large-vessel vasculitides. Overall, involvement of the CNS and peripheral nervous system (PNS) is common in primary systemic vasculitides. In contrast, such involvement is only occasionally observed in the setting of infectious diseases or malignancy or as a consequence of drug use. The pattern of PNS involvement in primary and secondary vasculitides is almost invariable, since it is related to occlusion of the vasa nervorum due to necrotizing small-vessel vasculitis. The ensuing picture is that of sensory axonal polyneuropathy or mononeuritis multiplex. The range of possible manifestations of CNS involvement is much wider, since several mechanisms can be implicated.

Original languageEnglish
Title of host publicationNeuroinflammation
PublisherElsevier Inc.
Number of pages27
ISBN (Print)9780123849137
Publication statusPublished - 2011

ASJC Scopus subject areas

  • Immunology and Microbiology(all)


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