Abstract
The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) based on immunofluorescence findings in kidney biopsies has provided insights into these two distinct diseases. C3G is further classified into dense deposit disease and C3 glomerulonephritis (C3GN) based on electron micrographic findings. Although these diseases have poor outcomes, limited Japanese literature confined to small, single-center cohorts exist on these diseases. We retrospectively analyzed 81 patients with MPGN type I and III from 15 hospitals in the Japan Renal Biopsy Registry to compare demographic, clinical characteristics and treatment outcomes of patients with IC-MPGN to those with C3GN. Of the 81 patients reviewed by immunofluorescence findings in kidney biopsies, 67 patients had IC-MPGN and 14 patients had C3GN. Age at diagnosis and systolic and diastolic pressure were higher and proteinuria and impaired renal function were significantly more prevalent in patients with IC-MPGN than those with C3GN. About 80% of the patients in both groups were treated with immunosuppressive therapy. At last follow-up (median 4.8 years), complete remission rate of proteinuria was significantly higher in patients with C3GN (64.3%) than in those with IC-MPGN (29.9%; P = 0.015). The renal survival rate was lower in patients with IC-MPGN when compared to C3GN (73.1% vs. 100%; log-rank, P = 0.031). Systolic blood pressure and renal function at baseline were independent predictors of progression to end-stage kidney disease. The overall prognosis of patients with C3GN is more favorable than for patients with IC-MPGN.
Highlights
Membranoproliferative glomerulonephritis (MPGN) has previously been used as an umbrella term to describe a spectrum of hypocomplementemic glomerular diseases, which are a rare cause of end stage kidney disease (ESKD)
This study presents the clinical and laboratory characteristics and clinical outcomes of patients form the largest Japanese immune-complex MPGN (IC-MPGN)/C3 glomerulonephritis (C3GN) cohort to date drawn from a multicenter, nationwide study based on the Japan Renal Biopsy Registry (J-RBR)
A patient’s baseline systolic blood pressure and renal function were independent predictors of progression to ESKD, whereas a patient’s baseline renal function and serum albumin were independent predictors of declining renal function (50% increase in serum creatinine (sCr) from baseline)
Summary
Membranoproliferative glomerulonephritis (MPGN) has previously been used as an umbrella term to describe a spectrum of hypocomplementemic glomerular diseases, which are a rare cause of end stage kidney disease (ESKD). MPGN has been reclassified into two diseases: immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) based on immunofluorescence findings in kidney biopsies: predominant or exclusive C3 deposits in C3G and combined immunoglobulins and complement deposits in IC-MPGN [1]. C3G is further classified into dense deposit disease and C3 glomerulonephritis (C3GN) based on electron micrographic findings. These updated classification criteria do not specify how best to clinically differentiate these two diseases in terms of diagnosis, optimal treatment, and prognosis. Recent criteria to differentiate C3G from IC-MPGN is based on C3 predominance, rather than immunoglobulins, in tissues with an otherwise MPGN-like pattern of glomerular disease using immunofluorescence tests [2]
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