Clinical characteristics of idiopathic membranoproliferative glomerulonephritis / 대한내과학회지

ABSTRACT

BACKGROUND: Idiopathic membranoproliferative glomerulonephritis (MPGN) is a chronic primary glomerular disease that occurs in both children and adults, with generally progressive course. We have examined the clinical and long-term outcome of patients with idiopathic MPGN at Keimyung University Dongsan Medical Center. METHODS: Of the total 1,971 patients with biopsy-proven glomerulonephritis over the 21-year period from June 1982 and June 2003, there were 51 cases of idiopathic MPGN of whom 49 had type I and two type II. RESULTS: Of the total 51 idiopathic MPGN, male to female ratio was 1.7:1, a mean age at diagnosis was 32 +/- 17 years (range; 6-70) and 50% of the patients were under the age of 30. The clinical presentations at the time of diagnosis were nephrotic syndrome (70%), asymptomatic urinary abnormality (18%), acute nephritic syndrome (6%), and recurrent gross hematuria (6%). Of the 40 patients who followed more than 6 months, with a mean follow-up of 71months, 10 patients progressed to end-stage renal disease. The renal survival at 5 and 10 years after diagnosis were 86 and 52%, respectively. Eight (20%) patients obtained a complete remission and none of them progressed to end-stage renal failure. The quantity of proteinuria at the time of biopsy was much more prominent in deteriorating group, though not significant (p=0.05) and young age and female seemed associated with the complete remission (p<0.05). CONCLUSION: Idiopathic MPGN remains a disease with a poor prognosis. Age, gender and quantity of proteinuria at the time of diagnosis were associated with the prognosis. Further prospective study with larger number of patients would be necessary to assess the prognostic factors and effective therapy for idiopathic MPGN.