Dense deposit disease: A variant of membranoproliferative glomerulonephritis

Habib, R; Gubler, Marie–Claire; Loirat, Chantal; Maı̈z, H. Ben; Lévy, Micheline

doi:10.1038/ki.1975.32

Cited by 189 publications

(131 citation statements)

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“…20,21 Previous studies have described the presence of increased neutrophils in otherwise typical membranoproliferative or mesangial proliferative lesions. 6,15,29 In addition, the diffuse endocapillary proliferation with neutrophilic infiltration seen in our study has been previously reported. 17 This pattern is easily mistaken for a postinfectious glomerulonephritis on light microscopy examination alone.…”

Section: Clinical Datasupporting

confidence: 85%

“…Alterations similar to those seen within the glomerular basement membrane were often present along tubular basement membranes and the basement membrane of Bowman's capsule, when examined. 9,24 The major finding from this international series of dense deposit disease cases is that the light microscopy pattern is variable and that the presence of a membranoproliferative pattern is seen in only one of four patients with dense deposit disease (similar to Habib et al above 6 ).…”

Section: Clinical Datasupporting

confidence: 59%

“…[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] In these reports, the histologic description of membranoproliferative glomerulonephritis II is usually a membranoproliferative pattern, although other light microscopy changes including a crescentic form and a mesangial proliferative form are included in some of the reports. [6][7][8]12,13,[15][16][17][20][21][22][23] However, the frequency, variability and definition of the light microscopy patterns present in patients with dense deposit disease are not apparent from previous reports. Thus, the purpose of this study was to examine a large number of renal biopsies from patients with dense deposit disease in order to characterize the light microscopic patterns seen in association with the diagnostic feature of dense deposit disease, electron dense transformation of the glomerular basement membranes.…”

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confidence: 99%

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Dense deposit disease is not a membranoproliferative glomerulonephritis

et al. 2007

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Section: Clinical Datasupporting

confidence: 85%

Section: Clinical Datasupporting

confidence: 59%

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confidence: 99%

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Dense deposit disease is not a membranoproliferative glomerulonephritis

et al. 2007

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“…The percentage of adult patients older than 60 yr in our study was 39%, compared with 0% in the 1975 French study by Habib et al, the 1975 French-Belgian study by Galle and Mahieu, the 1983 U.K. study by Cameron et al, and several other smaller studies (5,6,8,10,13,14). The reason for the larger representation of elderly patients in our study is unclear.…”

Section: Discussionmentioning

confidence: 41%

“…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).…”

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confidence: 99%

Dense Deposit Disease

Nasr

Valeri

Appel

et al. 2009

Clinical Journal of the American Society of Nephrology

156

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Background and objectives: Dense deposit disease (DDD) is a rare disorder that most commonly affects children. This study reports the largest North American series addressing clinicopathologic and outcome differences in children and adults.Design, setting, participants, & measurements: Thirty-two patients with DDD were analyzed from the archives of Columbia University between 1977 and 2007. Characteristic intramembranous electron-dense deposits defined all diagnoses.Results: The cohort included 14 children (<16 yr) and 18 adults, with 39% of adults >60 yr. The female/male ratio was 1.9. At presentation, the mean 24-h urine protein was 4.6 g, nephrotic syndrome was present in 33%, renal insufficiency in 59%, and hematuria in 87% of patients. Compared with adults, children had lower incidence of renal insufficiency and were more likely to have reduced C3. Histologic pattern included membranoproliferative, mesangial, endocapillary, and crescentic glomerulonephritis. Treatment included immunosuppression (IS) alone in seven, renin angiotensin system (RAS) blockade alone in six, and combined IS/RAS blockade in 11. On follow-up (mean 63 mo) available in 27 patients, 26% had complete response, 48% had persistent renal dysfunction, and 26% had ESRD. Correlates of ESRD were older age and higher creatinine at biopsy, the absence of combined IS/RAS blockade therapy and the presence of subepithelial humps, but not histologic pattern. On multivariate analysis, age and creatinine emerged as the only independent predictors of ESRD.Conclusions: DDD is clinically and pathologically heterogeneous. Adults have worse outcome than children, despite similar treatment. Combined IS/RAS blockade appears superior to either agent alone.

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C3 glomerulopathy: Understanding an ultra‐rare complement‐mediated renal disease

Heiderscheit

Hauer

Smith

2022

American J of Med Genetics Pt C

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C3 glomerulopathy (C3G) describes a pathologic pattern of injury diagnosed by renal biopsy. It is characterized by the dominant deposition of the third component of complement (C3) in the renal glomerulus as resolved by immunofluorescence microscopy. The underlying pathophysiology is driven by dysregulation of the alternative pathway of complement in the fluid-phase and in the glomerular microenvironment.Characterization of clinical features and a targeted evaluation for indices and drivers of complement dysregulation are necessary for optimal patient care. Autoantibodies to the C3 and C5 convertases of complement are the most commonly detected drivers of complement dysregulation, although genetic mutations in complement genes can also be found. Approximately half of patients progress to end-stage renal disease within 10 years of diagnosis, and, while transplantation is a viable option, there is high risk for disease recurrence and allograft failure. This poor outcome reflects the lack of disease-specific therapy for C3G, relegating patients to symptomatic treatment to minimize proteinuria and suppress renal inflammation. Fortunately, the future is bright as several anti-complement drugs are currently in clinical trials.

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Dense deposit disease: A variant of membranoproliferative glomerulonephritis

Cited by 189 publications

References 11 publications

Dense deposit disease is not a membranoproliferative glomerulonephritis

Dense deposit disease is not a membranoproliferative glomerulonephritis

Dense Deposit Disease

C3 glomerulopathy: Understanding an ultra‐rare complement‐mediated renal disease

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