Primary Focal Segmental Sclerosis Research Articles

Anti-Nephrin Antibody as a Potential Etiology in Primary Focal Segmental Sclerosis (FSGS)

Anti-Nephrin Antibody as a Potential Etiology in Primary Focal Segmental Sclerosis (FSGS)

Glomerular capillary C3 deposition as a risk factor for unfavorable renal outcome in pediatric primary focal segmental glomerular sclerosis.

Some patients with primary focal segmental sclerosis (FSGS) demonstrate complement 3 (C3) deposition in glomerular capillary loops (Cap-C3) and/or mesangial area (Mes-C3). The clinicopathological and prognostic significance of C3 deposition remains incompletely investigated, especially in the pediatric cohort. We retrospectively analyzed 264 children of biopsy-proven primary FSGS between January 2003 and December 2020. The correlation between Cap-C3 and renal outcome was evaluated by the Kaplan-Meier method and Cox multivariate regression analysis. Renal end-point event was defined as the development of end-stage renal disease, death for renal disease, or an estimated glomerular filtration rate reduction by at least 50% from baseline. Among the 264 patients, 30 (11.4%) had Cap-C3. Kaplan-Meier analysis showed that patients with Cap-C3 had significantly lower renal survival rates than patients without Cap-C3 (60.17% vs. 84.71% at 5 years, 39.49% vs. 65.55% at 10 years, P < 0.01). Cox multivariate regression analysis showed that Cap-C3 was an independent risk factor for poor renal outcome (HR 3.53, 95% CI 1.22-10.19, P = 0.02). Glomerular capillary C3 deposition was an independent risk factor for unfavorable renal outcome in children with primary FSGS.

Detection of Mature Macrophages in Urinary Sediments: Clinical Significance in Predicting Progressive Renal Disease

The ability to predict the rate of progression of renal parenchymal disease may help in its clinical management. We undertook characterization of urinary macrophages obtained from patients with various renal diseases paving special attention to the differentiation from non-progressive to progressive renal diseases. A total of 84 patients were divided into one of three categories. A highly progressive group included patients with rapidly progressive glomerulonephritis, diabetic nephropathy, membranoproliferative glomerulonephropathy, primary focal segmental sclerosis and diffuse proliferative lupus nephropathy, moderately progressive group included those with IgA nephropathy and Alport's syndrome and non progressive group included patients with thin basement membrane nephropathy, minimal change nephrotic syndrome, idiopathic renal hematuria and urolithiasis. Urinary sediments were reacted with four monoclonal antibodies (CD68/macrophages vimentin, cytokeratin, and 25F9/mature macrophages). In normal individuals mature macrophages (25F9+ cells) were absent in urinary sediments. The number of 25F9+ cells in the urine was highest in the highly progressive group, less prominent in the moderately progressive group, and virtually absent in the non progressive group. The 25F9+ cells reacted with anti-CD68 and anti vimentin antibody, whereas the 25F9+ cells did not react with anti-cytokeratin antibody. These findings indicate that the detection of mature macrophages in urine is useful to estimate the prognosis of renal parenchymal diseases and may help to differentiate some glomerular diseases (e.g., thin basement membrane disease vs. Alport's syndrome, and minimal change nephrotic syndrome vs. primary focal segmental sclerosis).