Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic...

We present a 77-year-old Caucasian woman who presented with nephrotic-range proteinuria, microhematuria, renal impairment, and extremely elevated blood pressure. She had a long history of well-controlled type 2 diabetes. Renal biopsy revealed fibrillary deposits in the mesangium and glomerular basement membrane consistent with fibrillary glomerulopathy (FGN), with crescentic changes and thrombotic microangiopathy (TMA). We could not identify any radiological, clinical, or laboratory evidence of autoimmune disorders, lymphoproliferative disorders, and malignancy. It was decided not to offer her any immunosuppressive therapy, as she was frail with substantial renal damage on the biopsy. Five months after presentation, she gradually progressed to requiring renal replacement therapy and is currently on maintenance hemodialysis. Crescentic changes in FGN, though rare, have been previously described, but the concurrent presence of TMA has never been previously reported.