Successful treatment of a unique case of congophilic fibrillary glomerulonephritis: A case report

INTRODUCTION: Amyloidosis and fibrillary glomerulonephritis (FGN) share similar electron microscopic signatures including random arrangement of fibrils. However, distinction between the 2 can often be made using Congo Red staining. PATIENT CONCERNS: Here we describe a unique case of FGN, which stained positive for Congo Red, as well as DnaJ heat shock protein family (Hsp40) member B9 which is more specific for FGN. The patient presented with acute kidney injury and severe proteinuria. DIAGNOSIS: Congophilic FGN. INTERVENTIONS: Six-month course of mycophenolate mofetil and prednisone. OUTCOMES: complete resolution of acute kidney injury and proteinuria TAKE HOME LESSONS: To our knowledge, this is the first reported case of successful treatment of this rare condition using mycophenolate mofetil and prednisone.