Crescentic glomerulonephritis: A Update on Pauci-immune and Anti-GBM diseases

A review focused on ANCA and anti-GBM nephritis

• Wegener's granulomatosis has been changed to granulomatosis with polyangiitis (GPA)
• A classification of ANCA nephritis has been proposed. It is based on % of glom with crescent and sclerosis.

• Vasculitis is seen most frequently in ANCA nephritis
• In addition to ANCA, LAMP-2 (lysosomal membrane protein) has been indicated as a cause of pauci-immune GN. However, the evidence is inconclusive.
• Autoimmune is indicated in pathogenesis of ANCA diseases. Genetic and environmental factors such as infection and drugs both have role.
• Again for anti-GBM, genetic and environment are working together to produce auto-antibody. The environmental triggers include organic solvent, viral infection and cigarette smoke.
• Dual disease (ANCA + anti-GBM) exists. The severity is equal to anti-GBM, but the risk of recurrence is as high as ANCA.