Details
| Original language | English |
|---|---|
| Pages (from-to) | 420-425 |
| Number of pages | 6 |
| Journal | American Journal of Kidney Diseases |
| Volume | 40 |
| Issue number | 2 |
| Publication status | Published - Aug 2002 |
| Externally published | Yes |
Abstract
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.
Keywords
- Crescentic glomerulonephritis, Cyclophosphamide, Fibrillary glomerulonephritis (FG), Immunosuppressive treatment, Severe limitation of renal function, Severe nephrotic syndrome
ASJC Scopus subject areas
- Medicine(all)
- Nephrology
Sustainable Development Goals
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In: American Journal of Kidney Diseases, Vol. 40, No. 2, 08.2002, p. 420-425.
Research output: Contribution to journal › Article › Research › peer review
}
TY - JOUR
T1 - Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge
AU - Blume, Cornelia
AU - Ivens, Katrin
AU - May, Philip
AU - Helmchen, Udo
AU - Jehle, Peter M.
AU - Keller, Frieder
AU - Grabensee, Bernd
AU - Riedel, Martin K.
PY - 2002/8
Y1 - 2002/8
N2 - 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.
AB - 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.
KW - Crescentic glomerulonephritis
KW - Cyclophosphamide
KW - Fibrillary glomerulonephritis (FG)
KW - Immunosuppressive treatment
KW - Severe limitation of renal function
KW - Severe nephrotic syndrome
UR - http://www.scopus.com/inward/record.url?scp=0036325697&partnerID=8YFLogxK
U2 - 10.1053/ajkd.2002.34548
DO - 10.1053/ajkd.2002.34548
M3 - Article
C2 - 12148119
AN - SCOPUS:0036325697
VL - 40
SP - 420
EP - 425
JO - American Journal of Kidney Diseases
JF - American Journal of Kidney Diseases
SN - 0272-6386
IS - 2
ER -