Focal segmental glomerulosclerosis (FSGS) is a type of glomerular disease (disease that affects the glomeruli) that can cause permanent kidney disease in children and adults. It affects kidney function by attacking the glomeruli, the tiny structures inside the kidneys where blood is filtered. The term glomerulosclerosis is used to describe the scarring or hardening of the tiny blood vessels inside the kidneys. Consequently, protein and sometimes red blood cells leak into the urine.
The most common clinical feature of FSGS is nephrotic syndrome, which is characterized by generalized edema (fluid in the body tissues that causes swelling), proteinuria, hypoalbuminemia (abnormally low levels of albumin, which is normally the most plentiful protein in the blood), and hyperlipidemia (high cholesterol).
Patients who have HIV may develop HIV-associated FSGS. In HIV-associated FSGS, renal (kidney) function deteriorates quickly, leading to end-stage renal disease (ESRD) within a few weeks to one year. ESRD is fatal unless treated with dialysis or kidney transplantation.
Since the introduction of highly active antiretroviral therapy (HAART), which is used to treat HIV/AIDS, patients are less likely to experience kidney failure when the viral load of HIV decreases.
Although the cause of HIV-associated FSGS remains unknown, researchers believe HIV itself may cause it, or the conditions may be genetically inherited because the disease primarily affects African American men.
For unknown reasons, men are more likely to develop HIV-associated FSGS. The ratio of men to women is 10 to one. Most patients with HIV-associated FSGS are African American men, with an average age of 33 years. HIV-associated FSGS is rare in Caucasians. Worldwide, more than 95% of HIV-associated FSGS patients are black. About 50% of patients suffering from HIV-associated FSGS are intravenous (injection) drug users and the remaining are homosexual or bisexual men, heterosexual contacts of infected persons, or HIV-infected children.
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