Mesangiocapillary glomerulonephritis: A long‐term study of 40 cases

Abstract

Forty cases of mesangiocapillary glomerulonephritis are reviewed for whom both light and electron microscopy and full clinical data were available. Immunofluorescence microscopy (IF) was performed on 23 cases and complement screening (CH50, C4 and C3) on 25 cases, with follow-up period of 5-22 y. The results of EM revealed 17 cases (42 per cent.) of Type I and 23 cases (58 per cent.) of Type II MCGN but only 52 per cent. of Type II cases were correctly identified by light microscopy. Epimembranous deposits were seen as frequently in Type II as in Type I (26 per cent. and 30 per cent.) and fragmentation of glomerular capillary basement membranes (GBM) was seen in 27 per cent. of Type I cases. Overall patient survival was 49 per cent. at 10 y and that of patients who presented with nephrotic syndrome was poor (39 per cent. at 10 y). Persistent hypocomplementaemia with C3 Nephritic Factor was present in 40 per cent.; the survival of these patients was less than those with normal complement levels (70 per cent. vs 100 per cent. at 5 y) and they were also more likely to develop renal failure. Renal failure was more likely to develop in those with a creatinine clearance of less than 100 ml/min at presentation and where the biopsy showed substantial crescents in greater than 20 per cent. of glomeruli. Mean CH50, C3 and C4 was lower in the hypocomplementaemic as compared to normocomplementaemic patients, and there were no differences between Type I and Type II. IF showed immunoglobulins and fibrin as well as C3 in both Type I and Type II cases. Our results support the concept of an immune-complex mediated phase in both types of MCGN, and we further suggest that (a) epimembranous deposits are common in both Type I and Type II and (b) cases with fragmentation of the GBM should be designated Type Ia.

Membranoproliferative Glomerulonephritis Type II (Dense Deposit Disease)

“…The more probable outcome is chronic deterioration of renal function leading to ESRD in approximately half of patients within 10 yr of diagnosis (22)(23)(24)(25). In some patients, rapid fluctuations in proteinuria occur with episodes of acute renal deterioration in the absence of obvious triggering events; in others, the disease remains stable for years despite persistent proteinuria.…”

Dense Deposit Disease

“…The disease has a Caucasian racial predominance and is most commonly seen in children and adolescents (5,6). Its natural history is variable, but approximately 50% of patients progress to ESRD within 8 to 10 yr (6,7). Animal and human studies indicate that the pathophysiologic basis of DDD is an uncontrolled systemic activation of the alternative complement pathway because of the presence of an autoantibody to C3 convertase, mutations in the factor H gene, or the presence of an autoantibody to factor H (4).…”

“…Although several studies of DDD were performed before 1990 (5)(6)(7)(8)(9)(10)(11)(12)(13)(14), there has been only a single study in recent years evaluating the clinical characteristics and course of the disease (15). Particularly little is known about the course in adults, and only one study published in 1983 compared the features of DDD in children and adults (6).…”

A Newer Approach to the Management of Membrano Proliferative Glomerulonephritis – Experience from A Tertiary Level Hospital, Bangladesh

“…But, these drugs are associated with various complications and ultimately progress to end stage renal disease 6 . MPGN is a rare variety of childhood nephrotic syndrome, which accounts 5-14% on renal biopsy series and its modalities of treatment and prognosis is also guarded 7,8 . Various studies showed that 52-90% patients developed end stage renal disease (ESRD) within 10-20 years 8 .…”

“…MPGN is a rare variety of childhood nephrotic syndrome, which accounts 5-14% on renal biopsy series and its modalities of treatment and prognosis is also guarded 7,8 . Various studies showed that 52-90% patients developed end stage renal disease (ESRD) within 10-20 years 8 . In 2011, the authors managed three cases of MPGN in the Department of Paediatric Nephrology, BSMMU with injection methyl prednisolone, oral cyclophosphamide along with oral prednisolone.…”