Introduction to, and classification of, the systemic vasculitides

This overview serves as an introduction to the systemic vasculitides, which are a group of heterogeneous disorders sharing a common pathophysiological mechanism leading to blood vessel inflammation and tissue necrosis. Our lack of understanding of the aetiology for most forms of vasculitis has resulted in the development of a classification system, which is primarily based on vessel size. Such a system assists in the grouping together of similar conditions for the purposes of multi-centre studies. Difficulties arise in classification of the vasculitides due to considerable overlap of clinico-pathological features; for example, microscopic polyangiitis (MPA), Wegenerʹs granulomatosis (WG) and Churg–Strauss syndrome (CSS) may all cause the identical renal lesion of necrotizing glomerulonephritis. The rationale for treatment often depends on the type of vasculitis and on the extent of organ involvement. Treatment may be similar for different types of disease. The lack of validated diagnostic criteria has, however, resulted in the application of classification criteria in their place, and has highlighted the limited usefulness of classification criteria in clinical practice. Classification systems should assist in the determination of therapy and prediction of outcomes, but have many limitations, which are discussed further in this review.