What is Focal Segmental Glomerulosclerosis (FSGS)?
Focal Segmental Glomerulosclerosis (FSGS) is a rare disease that attacks the kidney’s filtering system (glomeruli) causing serious scarring. FSGS is one of the causes of a serious condition known as Nephrotic Syndromefeatured by Proteinuria, edema, hypoproteinemia and hyperlipemia.
What does Focal Segmental Glomerulosclerosis (FSGS) mean?
Sclerosis means “scarring to the filters of kidneys, glomeruli”. The word “focal” is added because in FSGS, only some of the glomeruli become scarred. “Segmental” means that only some sections of the glomerulus becomes scarred, just parts of them.
So Focal Segmental Glomerulosclerosis means:
Focal = some
Segmental = sections
Glomerulo = of kidney filters (glomeruli)
Sclerosis = are scarred
The prognosis of Focal Segmental Glomerulosclerosis(FSGS) is poor. It is indicated by data that for patients with FSGS, the 5-year survival rate of kidneys is 70%, while the 10-year survival rate is 40%.
For Focal Segmental Glomerulosclerosis(FSGS) patients who can remit completely by treatment, the condition is stable; on the other hand, for FSGS patients who can not remit, the kidney function will worsen constantly, and most of the patients(30%~63%) will develop into Kidney Failure.
The prognosis of FSGS is mainly relates to the severity of the proteinuria, the degree of the pathological changes of the kidney and the response to the treatments. For example:
1. For FSGS patients with persistent Nephrotic Syndrome, the 10-year survival rate is only 45%, while for FSGS patients without Nephrotic Syndrome, the survival rate is over 90%.
2. For FSGS patients with severe hyperlipidimia, if the 24 h urine protein quantitation exceeds 10 g, the kidney function will decrease sharply, and it is called Malignant Focal Glomerulosclerosis. The prognosis of this type is very poor.
3. For patients with FSGS, the prognosis is poor, if the pathological change is accompanied by diffuse mesangial proliferation, obvious arteriolosclerosis and renal tubular interstitial infiltration of inflammatory cells and interstitial fibrosis changes.
4. For patients with FSGS, the prognosis is also poor, if the treatment of hormones has no effects, and the proteinuria and the other clinical symptoms can not remit.
After receiving a kidney transplant, patients with FSGS may show relapse of Nephrotic Syndrome. And pathological changes of focal segmental glomerulosclerosis will occur in the transplant kidney, and the incidence can reach up to 40%. The factors which can lead to the relapse of FSGS are large quantities of proteinuria, the sharp worsening of the condition and the occurrence of diffuse mesangial proliferation. In addition, if women with FSGS who have kidney damages want to conceive, they will easily suffer from pre-eclampsia and the worsening of kidney function.