Immunoglobulin A vasculitis nephritis: Current understanding of pathogenesis and treatment

Abstract

The clinical spectrum of immunoglobulin A vasculitis nephritis (IgAVN) ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome, rapidly progressive glomerulonephritis, or even renal failure. Clinical and experimental studies have shown a multifactor pathogenesis: Infection triggers, impaired glycosylation of IgA1, complement activation, Toll-like-receptor activation and B cell proliferation. This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.

Keywords: Henoch-Schoenlein purpura; Immunoglobulin A nephropathy; Immunoglobulin A vasculitis; Immunoglobulin A vasculitis nephritis; Vasculitis, glomerulonephritis.