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  • Vasculitides affecting the kidneys are typically associated with hematuria and proteinuria, frequently presenting as a rapidly progressive glomerulonephritis.

  • Glomerular injury occurs in the setting of the small vessel vasculitides, associated with antineutrophil cytoplasmic autoantibodies (ANCA), antiglomerular basement membrane antibodies (anti-GBM), or the presence of immune complex formation such as with Henoch–Schönlein purpura (HSP), cryoglobulinemic vasculitis, and systemic lupus erythematosus (SLE).

  • They may affect the kidneys alone, but are more frequently part of a multiorgan disease that may affect the skin, upper and lower respiratory tracts, and the musculoskeletal, gastrointestinal, and nervous systems.




Vasculitides are inflammatory diseases of the blood vessels, which are typically classified by their size (Figure 32–1 and Table 32–1). The following sections will provide an overview of the various kinds of vasculitides. The remainder of this chapter will focus on small vessel vasculitis (SVV) because of its association with glomerulonephritis. The International Chapel Hill Consensus Conference (CHCC) has developed one of the most widely used nomenclature systems which specifies the names and definitions for most forms of vasculitis.

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Table 32–1.12Names and definitions of vasculitis adopted by the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitis.

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