|Focal Segmental Glomerulosclerosis (FSGS)|
|Patient Information from The Center for Glomerular Disease at Columbia University|
|Focal segmental glomerulosclerosis or FSGS is a disease that causes urinary loss of protein and eventual scarring of the glomerular filters of the kidney. The terms focal and segmental refers to the pattern of damage in the kidney as seen under a microscope. Focal means that some of the glomeruli are scarred while others are normal. Segmental means that only a part of an individual glomerulus is damaged.
FSGS can develop as an isolated kidney disease (referred to as primary FSGS). In these cases, the cause of the disease is unknown. FSGS can also be due to other causes (referred to as secondary FSGS). In some of these cases, the disease may due to being born with too few glomeruli (ie. being born with a single kidney) or it may be associated with conditions that overwork the filters of the kidney such as with massive obesity. However, a wide variety of factors including infections, medications/toxins, chronic systemic diseases and other chronic kidney diseases can also result in the same pattern of disease. Rarely, the disease is inherited and much research is ongoing at the Glomerular Center at Columbia to understand the genes that may be responsible for the disease.
Primary FSGS affects individuals of all ages. It is an important cause of kidney problems in children. In adults, it is also increasingly seen. It occurs more frequently in black individuals compared to whites and is more frequent in males.
More specific treatment may be used to help alter the immune system and try to cure the disease. There are several treatments available. Steroids are often used initially for several months and up to 50% of individuals will improve with this medication. Other immunosuppression drugs may be used in addition to or instead of the steroids. These include a transplant medication, cyclosporine (and a related medication called tacrolimus) and a chemotherapy agent, cyclophosphamide. Some have recently tried a different type of immunosuppressant called mycophenolate mofetil (MMF). Each of these treatments have different side effects associated with them which should be discussed with the nephrologist. All can increase the risk of infections and this too must be discussed with the physicians caring for FSGS patients. .
In cases of FSGS that is secondary to other disorders, a major focus of therapy is treatment of the underlying disorder (such as weight loss or treatment of infections or other illness).