- 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. |Favorite Table|Download (.pdf)
Table 31–1. Names and Definitions of Vasculitis Adopted by the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis.
Large vessel vasculitis1
Giant cell arteritis
Granulomatous arteritis of the aorta and its major branches, with a predilection for the extracranial branches of the carotid artery. Often involves the temporal artery. Usually occurs in patients older than 50 years and often is associated with polymyalgia rheumatica.
Granulomatous inflammation of the aorta and its major branches. Usually occurs in patients younger than 50 years.
Medium-sized vessel vasculitis1
Necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules.
Arteritis involving large, medium-sized, and small arteries, and associated with mucocutaneous lymph node syndrome. Coronary arteries are often involved. Aorta and veins may be involved. Usually occurs in children.
Small vessel vasculitis1
Granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium-sized vessels, eg, capillaries, venules, arterioles, and arteries. Necrotizing glomerulonephritis is common.
Eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels, and associated with asthma and blood eosinophilia.
Necrotizing vasculitis with few or no immune deposits affecting small vessels, eg, capillaries, venules, or arterioles. Necrotizing arteritis involving small and medium-sized arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs.
Vasculitis with IgA-dominant immune deposits affecting small vessels, eg, capillaries, venules, or arterioles. Typically involves skin, gut, and glomeruli and is associated with arthralgias or arthritis.
Vasculitis with cryoglobulin immune deposits affecting small vessels, eg, capillaries, venules, or arterioles, and associated with cryoglobulins in serum. Skin and glomeruli are often involved.
Cutaneous leukocytoclastic angiitis
Isolated cutaneous leukocytoclastic angiitis without systemic vasculitis or glomerulonephritis.