Fibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterized by randomly oriented fibrillar deposits with a mean diameter of 20 nm. By immunofluorescence (IF), the deposits stain for IgG, C3, and κ and λ light chains, suggesting that the fibrils may be composed of antigen-antibody immune complexes. A recent major advance in our understanding of the pathogenesis of FGN resulted from the discovery that a major component of the fibrils is DNA-J heat-shock protein family member B9 (DNAJB9), and immunohistochemical staining for DNAJB9 now makes it possible to diagnose FGN in the absence of ultrastructural evaluation. FGN has a poor prognosis, treatment options are currently limited, and transplant recurrence is not uncommon.
No takes yet. Share an insight, caveat, or question.
Rosenstock et al. (2019) studied this question.
Synapse has enriched 4 closely related papers on similar clinical questions. Consider them for comparative context: