Focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome in children and adolescents, as well as a leading cause of kidney failure in adults. It is also known as "focal glomerular sclerosis" or "focal nodular glomerulosclerosis." It accounts for about a sixth of the cases of nephrotic syndrome. (Minimal change disease (MCD) is by far the most common cause of nephrotic syndrome in children: MCD and primary FSGS may have a similar cause.)
In children and some adults, FSGS presents as a nephrotic syndrome, which is characterized by edema (associated with weight gain), hypoalbuminemia (low serum albumin, a protein in the blood), hyperlipidemia and hypertension (high blood pressure). In adults it may also present as kidney failure and proteinuria, without a full-blown nephrotic syndrome.
Some general secondary causes are listed below:
- Glomerular hypertrophy/hyperfiltration
- Unilateral renal agenesis
- Morbid obesity
- Scarring due to previous injury
- Focal proliferative glomerulonephritis
- Toxins (pamidronate)
- Human immunodeficiency virus-associated nephropathy
- Heroin nephropathy
Focal segmental glomerulosclerosis may develop following acquired loss of nephron's from reflux nephropathy. Proteinuria is nonselective in most cases and may be in subnephrotic range(nephritic range <3.0gm/24hr) or nephritic range.
There are currently several known genetic causes of the hereditary forms of FSGS.
- Blood tests — cholesterol
- Kidney biopsy
- Salt restriction and diuretics, such as furosemide, for edema
- Antihypertensives (especially ACEIs) — if the blood pressure is too high but not so for it, it has another protective effect
- Treat present hyperlipidemia (e.g. statins, fibrates; although fibrates are contraindicated in renal failure)
- Aldosterone antagonist to decrease proteinuria and thus offer a degree of reno-protection
- Angiotensin II receptor antagonist
- Corticosteroids, such as prednisone — based on the clinical judgment of physician (no broad consensus/guideline)
- Cytotoxics, such as cyclophosphamide may be used to induce remission in patients presenting with FSGS refractory to corticosteroids, or in patients who do not tolerate steroids
- Plasmapheresis — blood cleansing using a machine to remove the patient's blood plasma and replacing it with donor plasma
- Vitamin E
- Fish oil
- Immunosuppressive drugs
- If none of the above works, the patient will require dialysis with possibly later transplantation of a new kidney that probably will be lost fast by FSGS due the FSGS agressor agent on the blood.
- American Kidney Fund: http://www.kidneyfund.org/
- National Kidney Foundation: https://www.kidney.org/
- Nephrotic Syndrome Study Network: http://www.neptune-study.org/
- NephCure Kidney International: https://nephcure.org/