Minimal change disease and idiopathic FSGS: manifestations of the same disease

Abstract

Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the key histological findings in patients with idiopathic nephrotic syndrome (INS). Although MCD and idiopathic FSGS are often considered to represent separate entities based on differences in their presenting characteristics, histology and outcomes, little evidence exists for this separation. We propose that MCD and idiopathic FSGS are different manifestations of the same progressive disease. The gradual development of FSGS in patients with non-remitting or relapsing INS has been well documented. Moreover, FSGS is the uniform result of substantial podocyte loss in animal models, and a common feature of virtually all progressive human glomerulopathies. As evidence suggests a common aetiology, the pathogenesis of MCD and idiopathic FSGS should be studied together. In clinical trials, idiopathic FSGS should be considered to represent an advanced stage of disease progression that is less likely to respond to treatment than the earlier stage of disease, which is usually defined as MCD.

Correlation of electrophoretic urine protein banding patterns with severity of renal damage in dogs with proteinuric chronic kidney disease

“…11 This is in contrast with the correlation of glomerular gel scores in cases with glomerular damage scores based on LM, which neared a weak correlation, and suggests that the glomerular protein banding pattern is more indicative of the degree of damage to the glomerular basement membrane ultrastructure than to changes and podocytes can result in altered protein filtration but might not be detectable on LM. 37,39 In contrast, in cases with marked glomerulosclerosis, the severity of proteinuria based on gel electrophoresis might be inconsistent with the severity of glomerular damage on LM because sclerotic glomeruli do not contribute significantly to urine production. 40 The weak to modest correlations of the tubular gel scores with component or composite TI damage scores were lower than correlations of sCr and tubular biomarkers with TI damage scores, as found in a previous study.…”

Synaptopodin is upregulated by IL-13 in eosinophilic esophagitis and regulates esophageal epithelial cell motility and barrier integrity

“…While regulation of cytoskeletal dynamics by SYNPO is likely critical for function of both organs, SYNPO has been primarily studied in the context of nephropathies (31)(32)(33). Idiopathic nephrotic syndrome is a clinical syndrome associated with a variety of kidney pathologies, including minimal change disease (MCD) (34), in which there is effacement of the foot processes of podocytes, leading to loss of epithelial integrity and increased permeability associated with proteinuria (35,36). Importantly, MCD can be successfully treated by immunosuppressive drugs, suggesting that immunologic responses are critical (23,37,38).…”

Apolipoprotein A-Ib as a biomarker of focal segmental glomerulosclerosis recurrence after kidney transplantation: diagnostic performance and assessment of its prognostic value - a multi-centre cohort study

“…Early post‐transplant relapse is mainly clinically detected by the sudden increase in proteinuria. The role of the biopsy at this stage is limited because the typical histological lesions are not present and appear later, at least 1 month after the increase in proteinuria, making diagnostic confirmation and early treatment difficult .…”

“…Regarding the requirement of a histological pattern of segmental glomeruloesclerosis for the diagnosis of idiopathic FSGS, Maas et aI. [.] proposed that idiopathic FSGS and minimal change disease (MCD), another INS without glomerular sclerosis and generally responsive to steroids, are two manifestations of the same disease, sharing the effacement of podocyte foot process.…”