Nephrotic syndrome is a group of symptoms including massive proteinuria defined as a daily loss of 3.5 gm or more of protein, hyperlipidemia, generalized edema, and hypoalbuminemia which results from renal pathology. Nephrotic syndrome is caused by several diseases including membranous glomerulonephritis, minimal change disease, and focal segmental glomerulosclerosis. FSGS is the most common cause of nephrotic syndrome in Hispanics and African-Americans.
Primary FSGS is idiopathic but the secondary form of the disease may occur in association with other conditions such as HIV or sickle cell disease.
Primary FSGS is idiopathic but the secondary form of the disease may occur in association with heroin abuse or interferon therapy.
On light microscopy (LM), the glomeruli that are affected demonstrate segmental sclerosis and hyalinosis while the unaffected glomeruli appear relatively normal.
Electron microscopy (EM) shows focal damage of visceral epithelial cells. This epithelial damage is a hallmark of FSGS. Furthermore, EM shows effacement of foot processes, similar to minimal change disease.
FSGS generally appears negative on immunofluorescence microscopy (IF) because the focal and segmental lesions may involve only a minority of the glomeruli. However, IgM, C1, and C3 may be present in the sclerotic areas and or in the mesangium.
Small amounts of blood can be seen on urinalysis.
The proteinuria includes all blood proteins including clotting factors, albumin, and immunoglobulin.
This disease has a relatively poor prognosis with many individuals progressing to chronic kidney disease, with more than half of the patients developing end-stage kidney disease in 10 years.
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