some = Focal sections of = Segmental Focal Segmental kidney filters = Glomerulo FSGS Glomerulosclerosis = { are scarred Sclerosis

How can research help? Research, which often requires patient participation, can lead to a better understanding of the causes, provide better diagnoses and more effective treatments, and ultimately help to find a cure. Be part of the discovery: By studying patients, researchers can more quickly unlock the mysteries of this disease. To get involved in research and stay informed, go to: Nephrotic Syndrome Study Network

www.Nephrotic-Syndrome- NEPTUNE is a part of the NIH Rare Diseases Clinical Research Network (RDCRN). Funding and/or programmatic support for this project has been provided by U54 Studies.org DK083912 from the NIDDK and the NIH Office of Rare Diseases Research (ORDR), the NephCure Foundation and the University of Michigan. The views expressed in written materials of publications do not necessarily reflect the official policies of the or call 1-866-NephCure Department of Health and Human Services; nor does mention by trade names, commercial practices or organizations imply endorsement by the U.S. Government. What is Focal and Segmental Glomerulosclerosis? Symptoms, Diagnosis and Treatment

Each person has Focal Segmental Glomerulosclerosis (FSGS) is a Your nephrologist may also recommend: two kidneys in rare disease that attacks the kidney’s filtering system • Diuretics and low salt diet help to control edema their lower back. (glomeruli) causing scarring of the filter. FSGS • A medication that blocks a hormone system called is one of the causes of a condition known as the renin angiotensin system (ACE inhibitor or ARB) Nephrotic Syndrome (NS). to control blood pressure or lower urine protein What are some symptoms of FSGS? • Anticoagulants to prevent blood clots FILTERS The kidneys continuously • Proteinuria – Large amounts of protein “spilling” • Statins to control cholesterol filter blood and produce into the urine urine to remove waste • Maintaining a healthy diet – Correct amounts products, salts and excess • Edema – Swelling in parts of the body, most of protein and fluid intake according to your fluid. noticeable around the eyes, hands and feet, that nephrologist’s recommendations can become painful • Exercising • Hypertension – High blood pressure • Not smoking BLOOD GLOMERULUS • Hypoproteinemia – Low blood protein • Vitamins IN Each kidney is made up • Hypercholesterolemia – High level of cholesterol What causes FSGS? of approximately one in the blood million tiny filters called Some cases of FSGS are "idiopathic" which means it “glomeruli.” Much as a How is FSGS diagnosed? occurs with no known cause. Others are due to genetic coffee filter keeps coffee A series of tests are ordered: mutations. Some cases are secondary to various medical grounds in, glomeruli • Urine: determine the amount of protein in conditions. Researchers are actively trying to learn more. FILTERED keep valuable cells and the urine BLOOD Who gets FSGS? OUT protein in the blood. • Blood: determine levels of protein, creatinine, Children and adults of all ages and races can get albumin, cholesterol and others FSGS. People of African ancestry have an increased URINE • “Glomerular Filtration Rate” (GFR): an estimate of risk of getting FSGS. In children, it is the second OUT kidney function most common cause of NS and the second leading When glomeruli become • Kidney ultrasound cause of kidney failure. damaged, proteins begin • Kidney Biopsy: Removal of a small portion of the What are the complications of FSGS? leaking into the urine kidney for examination under the microscope A high percentage of patients with FSGS do not respond (proteinuria). Proteinuria • Genetic testing may be helpful in some cases to steroids. Other treatment options are available to control causes fluid to accumulate in the body and prolonged How is FSGS treated? proteinuria but some carry significant side effects. FSGS can leakage can lead to kidney Currently there are no FDA approved treatments, but recur in some patients who receive a kidney transplant. damage and even failure. usually a steroid called prednisone or prednisolone, is given to control proteinuria. SINGLESINGLE GLOMERULUSGLOMERULUS