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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.

The classification of vasculitides is based on the predilection for injury of the different vascular beds (Table 31–1). The following sections provide an overview of the various groups of vasculitides. The remainder of this chapter will focus on the clinical aspects of small vessel vasculitis (SVV) because of their association with glomerulonephritis.

Table 31–1. Names and Definitions of Vasculitis Adopted by the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis.

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