Fibrillary glomerulonephritis: early diagnosis associated with steroid responsiveness.
Patients with fibrillary glomerulonephritis usually present with nephrotic proteinuria, microscopic hematuria, impaired renal function, and hypertension; 50% develop end-stage renal disease within a few years. There is no known effective therapy for fibrillary glomerulonephritis. We describe three patients with biopsy-proven fibrillary glomerulonephritis, in whom pathognomonic fibrillar deposits of 20-nm diameter were seen by electron microscopy. All patients had nephrotic syndrome and normal renal function at the time of diagnosis. They were treated initially with prednisone, 1 mg/kg body weight, then tapered individually according to the clinical course. Additional therapy consisted of an angiotensin-converting enzyme inhibitor and diuretics. Proteinuria disappeared in two of three patients after 9 and 12 months of therapy. In one patient, proteinuria was reduced from 7.49 mg/mg creatinine to 0.63 mg/mg creatinine after 6 months of treatment. The kidney function remained normal in all three cases. Two patients are now free of steroid therapy for 9 and 6 months. They show no signs of recurrence of kidney disease and have normal renal function without significant proteinuria. Steroid therapy in patients with biopsy-proven fibrillary glomerulonephritis, starting with prednisone, 1 mg/kg body weight, and tapered individually according to the clinical course, is a promising strategy. Early start of treatment in patients with preserved renal function seems to be crucial for a favorable outcome.
Dickenmann
Michael
M
Division of Transplantation Immunology and Nephrology, Institute for Pathology, University Hospital, Basel, Switzerland. mdickenmann@uhbs.ch
Schaub
Stefan
S
Nickeleit
Volker
V
Mihatsch
Michael
M
Steiger
Jürg
J
Brunner
Felix
F
D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000188:drug therapy*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007381:Intermediate Filament Proteins / Q000648:ultrastructure; D007678:Kidney Glomerulus / Q000473:pathology / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use*; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology
D000806:Angiotensin-Converting Enzyme Inhibitors; D000893:Anti-Inflammatory Agents; D004232:Diuretics; D005938:Glucocorticoids; D007381:Intermediate Filament Proteins; D011241:Prednisone