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Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge
Cornelia Blume1, Katrin Ivens, Philip May
1Klinik für Nephrologie und Rheumatologie, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany. hblume@cityweb.de
Insights
Fibrillary glomerulonephritis (FG) often leads to kidney failure. Treatment with corticosteroids and cyclophosphamide showed promising renal recovery in two patients with FG and crescentic glomerulonephritis.
Area of Science:
- Nephrology
- Immunology
Background:
- Fibrillary glomerulonephritis (FG) is a rare kidney disease with a poor prognosis, frequently progressing to end-stage renal disease.
- FG associated with crescentic glomerulonephritis presents a diagnostic and therapeutic challenge.
Observation:
- Two female patients (41 and 50 years old) with biopsy-proven necrotizing FG and crescentic glomerulonephritis were evaluated.
- Both patients presented with severe renal dysfunction, nephrotic syndrome, and hypertension.
- Other systemic and infectious causes were excluded through comprehensive testing.
Findings:
- Treatment with corticosteroids and cyclophosphamide resulted in significant renal function recovery within six months for both patients.
- One patient maintained stable renal function for 3.5 years post-treatment.
- The specific contribution of cyclophosphamide to FG course versus the crescentic component remains undetermined.
Implications:
- Cyclophosphamide may be a beneficial treatment for FG, particularly when combined with crescentic glomerulonephritis.
- These preliminary findings warrant further investigation through multicenter clinical trials to establish optimal therapeutic strategies for FG.
- Early intervention with immunosuppressive therapy could potentially alter the aggressive natural history of FG.
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.