Differential risk of remission and ESRD in childhood FSGS

Abstract

Focal segmental glomerulosclerosis (FSGS) is the leading cause of steroid-resistant nephrotic syndrome in childhood and the most common form of end stage renal disease (ESRD) from glomerular disease. In order to assess the risk of progression of children with primary FSGS and the impact of proteinuria remission status on disease progression, we undertook this study to describe a cohort of 60 children and adolescents from the Glomerular Disease Collaborative Network. Of the 60 patients included in the cohort, 58% were African American. Median age was 16 years. Proteinuria ranged from 1.0-24.0 g/day/1.73 m(2); 57% were hypertensive, and the median estimated glomerular filtration rate (eGFR) was 90.2 ml/min/1.73 m(2). Complete remission was achieved in 20%, partial remission in 33%, and 47% have not achieved remission during follow-up with all prescribed therapy. Only ACE-I/ARB therapy was predictive of proteinuria remission in multivariate analysis (hazard ratio [HR] 3.35; 95% confidence interval [CI] 1.42-7.92). Renal survival was much improved in patients with complete or partial remission compared with no remission in univariate analysis. In multivariate analysis comparing no remission status, complete remission was associated with a 90% decreased risk of ESRD (HR 0.10, 95% CI 0.01-0.79, p =0.03). In summary, proteinuria remission status is a valid predictor of long-term renal survival in children with FSGS.

Circulating Permeability Factors in Idiopathic Nephrotic Syndrome and Focal Segmental Glomerulosclerosis

“…Treatments that have been used include corticosteroids with or without cyclophosamide (1,2), cyclosporine (3), mycophenolate mofitil (MMF) (4), and rituximab (5,6). If proteinuria can be diminished by these agents or by nonspecific therapies such as angiotensin converting enzyme inhibitors (ACEIs), angiotensin receptor blockers (ARBs), and/or lipid lowering agents, progression of renal dysfunction is slowed (7,8). Rapid progression to ESRD is associated with an increased incidence of posttrans-plant recurrence of proteinuria and FSGS.…”

Discrepancy between Medical Evidence Form 2728 and Renal Biopsy for Glomerular Diseases

“…Private practices, hospitals, and academic centers throughout the southeastern United States send renal biopsies to the UNC Nephropathology Laboratory for evaluation. Patients whose renal biopsies result in a diagnosis in one of the GDCNЈs disease registries are contacted through their referring physicians, and longterm consent is obtained from them for inclusion in the registry and related research studies (13)(14)(15)(16)(17)(18)(19).…”

The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic steroid-resistant nephrotic syndrome: a systematic review

“…Outcome is related to the histopathological features of the disease on renal biopsy, especially the extent of chronic changes such as glomerulosclerosis, tubular atrophy and interstitial fibrosis. The majority of children with FSGS and persistent proteinuria develop chronic renal failure, 2,15 while overall those with MCNS have a generally favourable outcome.…”