A Nephrological View of the Classification of Vasculitis

Abstract

From the nephrological perspective, immune mediated vascular inflammation in the kidney most commonly results in a necrotizing and crescentic glomerulonephritis. Based on immunohistologic findings this form of glomerular injury is separable into three distinct categories which include: 1) direct antibody binding to the glomerular capillary wall (e.g. anti-glomerular basement membrane disease); 2) immune complex deposition (e.g. systemic lupus erythematosis); or 3) the paucity of immune deposits (e.g. Wegener’s granulomatosis and polyarteritis nodosa). Each of these immunopathologic categories of vascular inflammation is associated with serologic markers (e.g. anti-glomerular basement membrane antibodies in Goodpasture’s syndrome, and anti-DNA antibodies in lupus, etc.). It is the pauci-immune category of disease which is associated with anti-neutrophil cytoplasmic autoantibodies (ANCA) (1–5). From the nephrologic perspective, ANCA are important as a serologic marker of a spectrum of disease, as an aid in classification and nosology, and as a potential factor in disease pathogenesis (6–10).