Classification

Vasculitis may be primary or secondary to a separate pathology, the most common and important of which is infection. The differing terminology used in the classification of the primary systemic vasculitides remains confusing. In 1952, Zeek proposed the classification of the vasculitides according to vessel size (11). Subsequent classification schemes reflected the size of the predominantly affected blood vessels as well as the discovery of the antineutrophil cytoplasmic antibodies. The most recent classification schemes in common use are those developed by the American College of Rheumatology in 1990 (12) and the diagnostic criteria developed at the Chapel Hill Consensus Conference in 1994 (13) as shown in Table 2.

The clinical presentation and histopathology of the vasculitides may overlap. When one considers the potent immunosuppression required to treat the primary systemic vasculitides, it becomes clear that mimics of vasculitis and causes of secondary vasculitis must be excluded (Table 3).

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