Immunoglobulin-Negative DNAJB9-Associated Fibrillary Glomerulonephritis: A Report of 9 Cases

Fibrillary glomerulonephritis (FGN) was previously defined by glomerular deposition of haphazardly-oriented fibrils that stain with antisera to immunoglobulins and are negative for Congo red. We report the first series of immunoglobulin-negative FGN. The cohort consisted of 9 adults (7 females, 2 males), with a mean age at diagnosis of 66 years. Patients presented with proteinuria (100%, mean 3…