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Long-term clinical and morphological evaluation of primary membranoproliferative glomerulonephritis
1Section of Nephrology, Department of Internal Medicine, Taichung Veterans General Hospital, Taiwan, R.O.C.
Background:
Membranoproliferative glomerulonephritis (MPGN) is a relatively rare primary glomerulonephritis (GN). Its incidence has decreased progressively in the past two decades. To improve knowledge of this rare GN, a retrospective review of 22 patients during a 12-year period was undertaken.
Methods:
From November 1982 to December 1994, from a total 814 cases of primary GN, 22 patients with primary MPGN were diagnosed. Clinical data, medical records, renal pathology and outcome were reviewed.
Results:
Patients included 15 male and 7 females, aged from 11 to 67 years. The average follow-up period was 46.3 months, with a range of 1 to 140 months. Tissue was available for electromicroscopic study in 11 cases; of which 9 cases fulfilled morphologic criteria of Type I MPGN; the other 2 cases were Type II MPGN. The clinical presentations at diagnosis included nephrotic syndrome (86.4%), impaired renal function (63.6%), microhematuria (50%), gross hematuria (31.8%) and hypertension (50%). Low serum C3 was found in 40.9% cases, 44.4% in Type I and 50% in Type III MPGN. The positive rate of hepatitis B virus infection was 22.7% with 33.3% in Type I and none in Type III MPGN. All 22 patients received various combined antihypertensive agents, immunosuppressant, anticoagulant and antiplatelet agents at diagnosis, but 17 had progressive disease, 4 maintained normal renal function with proteinuria and only 1 had complete remission. Fifteen patients, including six Type I and no Type III MPGN, progressed to end-stage renal failure. Both patients with Type III MPGN maintained normal renal function and responded to treatment. The 5 and 10 year actuarial renal survival rates were 33.3% and 16.7% respectively. The median kidney survival time was 51.2 months.
Conclusions:
A majority of cases with MPGN presenting with impaired renal function (86.7%) and hypertension (85%) at diagnosis progressed to end-stage renal failure. Delayed diagnosis and poor compliance were possible reasons for compared with for worse prognosis previous reports. But two patients with Type III MPGN had favorable prognosis previously described. Treatments generally failed to halt disease progression. Since at least five cases (22.7%) maintained normal renal function after treatments, a course of immunosuppressant is probably indicated if there is no contraindication. Further study with a larger population is warranted to clarify this issue.
Insights
Membranoproliferative glomerulonephritis (MPGN) often leads to kidney failure, especially when diagnosed with impaired renal function or hypertension. Immunosuppressants may offer benefit in select cases, warranting further study.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- Membranoproliferative glomerulonephritis (MPGN) is a rare primary glomerulonephritis.
- Its incidence has declined over the past two decades.
- This study aimed to improve understanding of this rare condition through a retrospective review.
Purpose of the Study:
- To analyze the clinical characteristics, pathology, and outcomes of primary MPGN.
- To evaluate the effectiveness of various treatments on disease progression.
- To identify factors influencing prognosis in MPGN patients.
Main Methods:
- Retrospective review of 22 primary MPGN patients diagnosed between 1982 and 1994.
- Analysis of clinical data, medical records, and renal pathology, including electromicroscopic studies.
- Review of patient outcomes, including renal survival rates and response to treatment.
Main Results:
- Nephrotic syndrome, impaired renal function, and hypertension were common at diagnosis.
- Type I MPGN was more prevalent than Type II.
- Fifteen patients progressed to end-stage renal failure; 5 cases maintained normal renal function post-treatment.
- Type III MPGN showed a favorable prognosis.
Conclusions:
- MPGN with impaired renal function or hypertension at diagnosis often progresses to end-stage renal failure.
- Current treatments were largely ineffective in halting disease progression.
- Immunosuppressive therapy may be beneficial for some MPGN patients, particularly those with Type III, necessitating further investigation.