What is Nephrotic Syndrome?

Nephrotic syndrome (NS) is not a disease, but an umbrella term for the collection of signs and symptoms that occurs when there is a problem with the kidney’s filtering system (glomeruli) and causes bloodstream proteins to leak into the urine (proteinuria).

Nephrotic syndrome has many causes. The most common are:

Icon of a multi-colored umbrella representing the types of nephrotic syndrome

Common symptoms

  • Swelling in parts of the body (edema) most visible in the head, hands, feet and belly
  • Low level of protein in the blood (hypoalbuminemia)
  • High blood cholesterol (hypercholesterolemia)
  • High blood pressure (hypertension)

What causes it?

‍‍The specific cause of nephrotic syndrome can only be made with a kidney biopsy. Children, specifically, may be treated with steroids instead of having a biopsy, if there are no other complications.

Who gets it?

Anyone can get nephrotic syndrome.

Nephrotic syndrome is one of the most common causes of chronic kidney disease and is responsible for about 12% of kidney failure in adults and 20% in children.

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Focal Segmental Glomerulosclerosis (FSGS)

Focal Segmental Glomerulosclerosis (FSGS) is a disease that affects the kidney’s filtering system (glomeruli) causing scarring and loss of large amounts of protein in the urine.

Who gets it?

Children and adults of all ages and races can get FSGS

Common symptoms

FSGS causes loss of protein in the urine which may lead to:

  • Swelling in parts of the body (edema) most visible in the head, hands, feet and belly
  • Low level of protein in the blood (hypoalbuminemia)
  • High blood cholesterol (hypercholesterolemia)
  • High blood pressure (hypertension)
  • Kidney failure

What causes it?

There is no single cause of FSGS. When the cause is not known it is called idiopathic or primary FSGS.

In some patients FSGS may be the result of a genetic or inherited disease, an infection, or another kidney disease that leads to scarring.

What is the treatment?

Very few treatments are available for patients with FSGS. Most often, patients are treated with drugs called steroids (prednisone or prednisolone) and with blood pressure medicines. Some patients respond well to other medicines that suppress the immune system. To date, there is no treatment that works for every person with FSGS.

Patients who do not respond to treatments are at risk for kidney failure. The NEPTUNE study aims to find out more about the disease for better treatments.

How is it diagnosed?

With information obtained from blood tests, urine tests, and a kidney biopsy, a physician can determine if a person has FSGS.

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Minimal Change Disease (MCD)

Minimal change disease can occur at any age but is the most common cause of nephrotic syndrome in children. Minimal change disease is the diagnosis given when a patient has nephrotic syndrome and a kidney biopsy shows little or no change to the structure of the kidney filters (glomeruli) or the rest of the kidney tissue. Tiny drops of a fatty substance called a lipid may be present, but no scarring has taken place within the kidney.

Who gets it?

People of all ages can get minimal change disease, though it mostly affects young children in the pre-school age group.

Common symptoms

The most common symptoms are swelling around the eyes, face, belly, and legs. A person with minimal change disease may make less urine, gain weight, and become swollen during active phases of the disease.

What causes it?

The cause is not known but researchers are actively trying to learn more.

What is the treatment?

Usually the doctor will prescribe a drug called prednisone or prednisolone. This drug helps to stop the loss of protein in the urine and increase the amount of urine expelled from the body. Most patients will improve on this drug after several weeks of treatment.

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Membranous Nephropathy (MN)

Membranous nephropathy is a kidney disease that is caused by a build up of proteins in the part of the kidney filter called the glomerular basement membrane. This part of the glomerulus is the thin tissue that helps separate the blood from the urine. In membranous nephropathy, the basement membrane becomes thick and damaged, allowing large amounts of protein to leak out of the blood and into the urine.

Who gets it?

Anyone can have membranous nephropathy, but it is more common in adults over the age of 40.

Common symptoms

Symptoms occur slowly and can include swelling, weight gain, and high blood pressure.

What causes it?

The cause is not known but researchers are actively trying to learn more.

What is the treatment?

For people with large amounts of protein in the urine, medications to suppress the immune system are often used. These include corticosteroids, cyclosporine, tacrolimus, and cyclophosphamide.

Limiting salt in the diet and using medications to help the body get rid of extra salt and water are often helpful. Blood pressure medications that can decrease protein in the urine are also used.

Sometimes medicines are prescribed to decrease the chance of blood clots, which can be a problem in patients with nephrotic syndrome and membranous nephropathy. The NEPTUNE study aims to understand membranous nephropathy to develop better treatments.

How is it diagnosed?

With results from blood tests, urine tests and, most importantly, a kidney biopsy, a doctor can determine if a person has membranous nephropathy.

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