Focal segmental glomerulosclerosis (FSGS) is a disease in which scar tissue develops on the parts of the kidneys that filter waste out of the blood (the glomeruli). FSGS can be caused by a variety of conditions. Without treatment, primary FSGS will usually lead to kidney failure where the only treatment options are dialysis or kidney transplant.
Signs and symptoms
The most common clinical presenting feature of FSGS (>70% of patients) is nephrotic syndrome, characterized by generalized edema, massive proteinuria, hypoalbuminemia, and hyperlipidemia. However, the natural history of FSGS is variable and can range from edema that is difficult to manage, to proteinuria that is refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and a progressive loss of renal function.
In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESRD. In human immunodeficiency virus (HIV)–associated FSGS, the renal functional deterioration is rapid, leading to ESRD within a few weeks to 1 year.
Diagnosis For FSGS
Kidney (renal) function testing. Blood tests and an analysis of a 24-hour urine collection are mainstays. Sometimes a more sophisticated test may be ordered — an iothalamate clearance test, which uses a special contrast agent to track how well your kidneys are filtering. Mayo Clinic is one of the few centers to specialize in iothalamate clearance testing using a dedicated renal pathology laboratory.
Kidney biopsy. Biopsy is the most accurate test to confirm the diagnosis of many kidney diseases, including FSGS. Because of the large number of people they evaluate each day, Mayo Clinic specialists are very experienced with kidney biopsies and have an outstanding track record for safety.
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