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Mesangiocapillary glomerulonephritis type II (dense-deposit disease): clinical features of progressive disease
W M Bennett1, R G Fassett, R G Walker
1Department of Medicine, Oregon Health Sciences University, Portland 97201.
Abstract:
Twenty-seven patients presenting to the Royal Melbourne Hospital between 1968 and 1988 with mesangiocapillary glomerulonephritis type II with intramembranous dense deposits (dense-deposit disease, DDD) are analyzed. Patients were divided into two groups on the basis of whether renal function deteriorated (14 patients) or remained stable (13 patients). At presentation or during the course of the disease, heavy proteinuria, macroscopic hematuria, and high quantitative urinary red cell or white cell counts characterized patients with progressive disease. Patients with crescents on their initial renal biopsy or with large numbers of polymorphs in glomerular capillaries corresponding with sterile pyuria were more likely to have deterioration of renal function. The average time from onset of symptoms to development of end-stage renal disease was over 16 years. The patient's clinical course could not be anticipated by serum complement profiles, the presence of C3 nephritic factor, or partial lipodystrophy. Pregnancy did not affect the course of the disease. Six patients underwent renal transplantation and the disease recurred on renal biopsy in four. However, only two individuals lost renal allografts due to recurrent DDD.
Insights
Dense-deposit disease (DDD) progression is linked to proteinuria, hematuria, and specific biopsy findings. While not predictable by complement levels, DDD can recur after kidney transplantation.
Area of Science:
- Nephrology
- Pathology
Background:
- Mesangiocapillary glomerulonephritis type II, also known as dense-deposit disease (DDD), is a rare kidney disorder.
- Characterized by intramembranous dense deposits in the glomerular basement membrane, DDD can lead to progressive renal dysfunction.
Purpose of the Study:
- To analyze the clinical course and prognostic factors of dense-deposit disease (DDD).
- To identify predictors of renal function deterioration in patients with DDD.
Main Methods:
- Retrospective analysis of 27 patients with DDD treated between 1968 and 1988.
- Correlation of clinical presentation, renal biopsy findings, and laboratory data with disease progression.
Main Results:
- Progressive renal disease was associated with heavy proteinuria, macroscopic hematuria, and sterile pyuria.
- Renal biopsy findings such as crescents and polymorph infiltration predicted deterioration.
- The average time to end-stage renal disease was over 16 years.
- Serum complement profiles, C3 nephritic factor, partial lipodystrophy, and pregnancy did not predict disease course.
Conclusions:
- Early indicators of progressive dense-deposit disease (DDD) include specific urinary and biopsy findings.
- Despite potential recurrence after transplantation, DDD does not invariably lead to graft loss.