Atypical anti-GBM nephritis

Patients with classic form of anti-GBM nephritis presents with rapidly progressive glomerulonephritis (RPGN) in which the renal function deteriorates rapidly and renal histology shows crescentic glomerulonephritis (>50% glomeruli with crescents). Nasr et al. reported a series of patients with atypical anti-GBM nephritis. These patients had milder degree of renal dysfunction but with more pronounced proteinuria. Immunofluorescence study showed linear staining of GBM with IgG in most cases but the light microscopy showed varied glomerular lesions with or without small number of crescents. Half of the patients showed monotypic IF staining though underlying plasma cell dyscrasia were not present.

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.