Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge.
Most cases of fibrillary glomerulonephritis (FG) terminate in end-stage renal disease within a few years. We report on two female patients (41 and 50 years old) with the diagnosis of FG associated with crescentic glomerulonephritis, a combination found in 20% to 25% of cases of FG. A broad spectrum of infectious disease and systemic immunologic disorders could be ruled out by specific assays. Both patients had severely impaired renal function, nephrotic syndrome, and hypertension. Based on the biopsy finding with necrotizing FG, treatment was started with corticosteroids and cyclophosphamide. In both patients, renal function recovered markedly within 6 months of treatment, in one case remaining stable for 3.5 years. Whether or not cyclophosphamide treatment changed the course of the FG itself or counteracted the acute crescentic process cannot be determined from these two patients. Based on these promising preliminary findings and the poor prognosis of FG, however, we recommend cyclophosphamide treatment of patients with FG and additional crescentic glomerulonephritis. For a systematic evaluation of the therapeutic options in FG, a multicenter clinical trial should be conducted.
Blume
Cornelia
C
Klinik für Nephrologie und Rheumatologie, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany. hblume@cityweb.de
Ivens
Katrin
K
May
Philip
P
Helmchen
Udo
U
Jehle
Peter M
PM
Riedel
M
M
Keller
Frieder
F
Grabensee
Bernd
B
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D006973:Hypertension / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000187:drug effects / Q000503:physiopathology; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide