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Haroon Khan

, David G. I. Scott

, and Richard A. Watts

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date: 27 January 2022

The initial description of one of the conditions that are now recognized as comprising the systemic vasculitides was provided by Heberden in the eighteenth century, followed by others by Schönlein, Henoch, Kussmaul, and Meier, in the nineteenth century. During the twentieth century, a number of other entities were described, including granulomatosis with polyangiitis (Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome), and microscopic polyangiitis. Most classification systems are based on a division by vessel size—large, medium, and small—a notion introduced by Zeek. The two major developments were the elaboration of the American College of Rheumatology criteria for the classification of the seven types of vasculitis, in 1990, and the Chapel Hill Consensus Conference, held in 1994 and which provided clear definitions for each condition. In this chapter, ten papers which illustrate these seminal developments in the field of vasculitis are presented within their context.

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