The vasculitides

This chapter presents a discussion on vasculitides. The vasculitides are a collection of diseases sharing the central feature of tissue injury due to inflammation of blood vessels. Clinical features, acuity of presentation, chronicity, or recurrences of inflammation depend upon the etiology, immunopathogenesis, and target organ systems. Peripheral or central nervous system features comprise a prominent part of many of these disorders. The classification of the vasculitides is evolving. There is no simple way to group the vasculitides that have neurological manifestations; however, one that is useful when discussing the diseases in terms of therapeutic approaches are illustrated. In vasculitis, the predominant mediator of tissue injury is ischemia. Depending on the size, distribution, and density of the vessels involved, the clinical features vary. In the central nervous system, encephalopathies, hemorrhages both subarachnoid and intraparenchymal, thrombotic strokes, seizures, cranial neuropathies, and myelopathies occur. Inflammatory cells binding to and passing through the blood vessel wall are a vital physiological process central to infection control and tumor surveillance functions of the immune system. The underlying sequential expression of cell surface molecules in response to proinflammatory stimuli is normally a well-regulated, self-limited process. The chapter discusses general clinical aspects of neurological symptoms in vasculitides, general principles of diagnosis, general principles of treatment, and others. The discussion also includes immune complex-mediated vasculitis, antibody-mediated vasculitis, t-cell-mediated vasculitis, and nonspecific vascular injury. Plasmapheresis may be effective therapy in vasculitis when circulating autoantibodies or immune complexes contribute to the pathogenesis of the vasculitis