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Long-term follow-up of atypical membranoproliferative glomerulonephritis: are steroids indicated?
Teruo Fujita1, Kandai Nozu, Kazumoto Iijima
1Department of Pediatrics, Kobe University Graduate School of Medicine, 6500017 Kobe, Hyogo, Japan. teruo@dream.ocn.ne.jp
Abstract:
Atypical membranoproliferative glomerulonephritis (MPGN) has been reported to have a good prognosis when treated with corticosteroids. However, this recommendation is based on uncontrolled trials and is associated with many complications. The purpose of our study is to determine whether steroid therapy is indicated for atypical MPGN. The cases of seven patients with atypical MPGN are reported in this study. Urinary abnormalities of five of them were detected by urine screening at school, of two because of macrohematuria. Hypocomplementemia was noted in six patients. All but one patient were treated without corticosteroids, and five with angiotensin-converting enzyme inhibitors (ACEI) and/or the Chinese herbal medicine Sairei-to (TJ-114). One patient recovered spontaneously from proteinuria and was therefore not treated, and one who developed severe proteinuria during observation was treated with corticosteroids. After an average follow-up period of 10.0 years, five patients showed normal urinary findings, one had hematuria and one proteinuria. At the most recent follow-up, the renal function of all patients remained within the normal range, and serum C3 had returned to normal levels in five out of six. These findings suggest that the indication of steroid therapy for atypical MPGN should be re-examined, since most of the patients with atypical MPGN seem to have an excellent prognosis without treatment with corticosteroids.
Insights
Corticosteroid treatment for atypical membranoproliferative glomerulonephritis (MPGN) may not be necessary. Most patients with atypical MPGN show excellent prognoses and normal renal function without steroid therapy, suggesting a need to re-evaluate treatment guidelines.
Area of Science:
- Nephrology
- Immunology
- Internal Medicine
Background:
- Atypical membranoproliferative glomerulonephritis (MPGN) is often treated with corticosteroids.
- This treatment recommendation is based on limited evidence from uncontrolled trials and carries risks of complications.
Purpose of the Study:
- To investigate the necessity of steroid therapy for atypical MPGN.
- To evaluate the long-term prognosis of patients with atypical MPGN treated without corticosteroids.
Main Methods:
- Retrospective case series of seven patients diagnosed with atypical MPGN.
- Treatment approaches included observation, angiotensin-converting enzyme inhibitors (ACEI), and the Chinese herbal medicine Sairei-to (TJ-114).
- One patient received corticosteroid treatment due to severe proteinuria.
Main Results:
- After a median follow-up of 10.0 years, five patients achieved normal urinary findings.
- All patients maintained normal renal function.
- Serum C3 levels normalized in five of six patients with initial hypocomplementemia.
Conclusions:
- The findings challenge the routine use of corticosteroids for atypical MPGN.
- Most patients with atypical MPGN appear to have an excellent prognosis without steroid therapy.
- Re-evaluation of current treatment guidelines for atypical MPGN is warranted.
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