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 Syndrome featured 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 i s 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.
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