Systemic vasculitides

Abstract

Systemic vasculitis is defined as widespread vascular inflammation, which may be a primary condition or consequent to another pathologic process. These syndromes represent a wide and varied array of entities, the great majority of which are poorly understood in terms of their underlying pathogenic mechanisms and most appropriate and effective therapies. In this chapter, we will give a brief overview of the systemic vasculitides, excluding those directly caused by an infective agent. The vasculitides are a heterogenous group of diseases with poorly understood pathophysiology, making their classification a somewhat artificial and arbitrary attempt to segment entities along what is likely a continuous spectrum. The syndromes are discussed according to the predominant size of the vessels it affects using the recently revised Chapel Hill Consensus Conference Nomenclature of Vasculitides standards.