Nephrotic Syndrome

Topic Overview

Illustration of the urinary system

What is nephrotic syndrome?

Nephrotic syndrome is a sign that your kidneys are not working right. You have nephrotic syndrome if you have high levels of protein in your urine, low levels of protein in the blood, and high cholesterol.

Nephrotic syndrome is not a disease. It is a warning that something is damaging your kidneys. Without treatment, that problem could cause kidney failure. So it’s important to get treatment right away.

Nephrotic syndrome can occur at any age. But it is most common in children between the ages of 18 months and 8 years.

What causes nephrotic syndrome?

There are tiny blood vessels in the kidneys that filter waste and extra water from the blood. When these filters are damaged, you get nephrotic syndrome. Protein helps move water from the tissues into the blood. Healthy kidneys keep the right amount of protein in the blood. Damaged kidneys let protein slip from the blood into the urine. Without enough protein in the blood, fluid builds up in the tissues. This can cause swelling.

Many things can cause this blood vessel damage, including diabetes, lupus, infection, certain cancers, and some medicines. Sometimes doctors don't know what causes it.

A type of kidney disease called minimal change disease (also called nil disease) causes most of the cases of nephrotic syndrome in children. Doctors don't know what causes minimal change disease.

What are the symptoms?

The most common early symptom of nephrotic syndrome, in both children and adults, is swelling in the tissues around the eyes or in the feet or ankles. They may also have swelling in the lungs that can make it hard to breathe.

But many people who have nephrotic syndrome don't have symptoms.

How is nephrotic syndrome diagnosed?

Doctors diagnose nephrotic syndrome using blood and urine tests.

You may have other tests to see what is causing nephrotic syndrome. Adults may also have a kidney biopsy, in which the doctor takes a sample of tissue for testing.

How is it treated?

Treatment focuses on reversing, slowing, or preventing further kidney damage. The treatment you need depends on whether you are an adult or a child and what health problem caused nephrotic syndrome. You may take medicine, and your doctor may suggest a diet that is low in salt and protein.

With treatment, young children usually get better and have no lasting problems. Often treatment is not as successful in older children and adults. If the kidney damage is not stopped, it can lead to chronic kidney disease.

Frequently Asked Questions

Learning about nephrotic syndrome:

Being diagnosed:

Getting treatment:

Living with nephrotic syndrome:

Cause

Nephrotic syndrome is caused by damage to the tiny blood vessels in the kidney that filter waste and excess water from the blood.

Many conditions and diseases can cause nephrotic syndrome, including:

  • Minimal change disease (also called nil disease), a type of kidney disease. The cause of minimal change disease is unknown. But this disease causes most of the cases of nephrotic syndrome in children.
  • Membranous glomerulopathy and focal segmental glomerulosclerosis, which are two other diseases of the kidney. Both of these diseases affect the glomeruli. The glomeruli help filter waste out of your blood. In focal segmental glomerulosclerosis, some of the parts of the glomeruli have scar tissue. This can affect how they filter the blood. In membranous glomerulopathy, the tissue wall (membrane) that separates blood and urine and acts as a filter in your kidney becomes thickened and damaged.
  • Diabetes and lupus. Diabetes is the most common cause of nephrotic syndrome in adults in the United States.
  • Infections, such as HIV, hepatitis B, hepatitis C, syphilis, malaria, tuberculosis, or post-streptococcal glomerulonephritis.
  • Cancer, such as breast cancer, lung cancer, Hodgkin's lymphoma, or multiple myeloma.
  • Medicines, such as nonsteroidal anti-inflammatory drugs, penicillamine, gold therapy, or captopril.
  • Illegal drugs, such as heroin.
  • Conditions such as preeclampsia, chronic graft rejection following an organ transplant, and allergic reactions to bee stings.
  • Unknown (idiopathic) factors.

Symptoms

Many people who have nephrotic syndrome do not have any noticeable physical symptoms. When symptoms are present, they can include:

  • Swelling in the tissues around the eyes (periorbital edema) or in the feet or ankles (peripheral edema). This is the most common early symptom of nephrotic syndrome in both children and adults.
  • Shortness of breath caused by fluid buildup in the lungs (pulmonary edema).
    • Adults older than 65 may be misdiagnosed with heart failure.
    • Children are often thought to have allergies.
  • Dry skin.
  • Swelling of the scrotum (scrotal edema), which may cause a cord in the testicles to twist (testicular torsion).

Signs of nephrotic syndrome in blood and urine tests can include:

What Happens

Nephrotic syndrome occurs when the kidneys are not working properly. Healthy kidneys filter out excess water, salts, and other items from the blood in our body. Large amounts of protein and minerals are lost through urine when kidneys have damaged filters. The body is left without enough protein to soak up water. As a result, the water moves from the blood supply into body tissues. This causes swelling in the tissues where the water pools.

The most common areas of swelling are in the face around the eyes and in the ankles and feet. Fluid can also collect in the lungs, making it difficult to breathe.

Complications of nephrotic syndrome can include:

Acute nephrotic syndrome can develop quickly over a few days to a few weeks, causing edema (swelling) and possibly kidney failure.

If another severe medical condition (such as diabetes or high blood pressure) is causing nephrotic syndrome, you may develop complications from the other condition as well.

Most children who have nephrotic syndrome do well with treatment and have a normal life expectancy. Children older than age 12 at the time of diagnosis and adults who also have diabetes or high blood pressure do not respond as well to treatment as do children younger than 12.

Complete recovery is possible. Doctors define complete recovery as living without symptoms or treatment for more than 2 years.

What Increases Your Risk

Diabetes is the most common cause of nephrotic syndrome in adults in the United States. About one-third of children who develop nephrotic syndrome have diabetes.

You also have an increased risk of developing nephrotic syndrome if you have a disease that results in an impaired immune system, such as AIDS or lupus.

Nephrotic syndrome is a relatively rare disease that can occur at any age.

When To Call a Doctor

Call 911 or other emergency services if you have nephrotic syndrome and you develop:

Call your doctor immediately if you have:

  • Bloody urine (gross hematuria).
  • Sudden, severe swelling in your legs, and pain when you flex your feet.
  • Swelling of the scrotum.
  • Sudden, severe increase in swelling around the eyes or in the hands, legs, or feet.
  • Sudden weight gain, such as 2 lb (0.9 kg) in 24 hours or 5 lb (2.3 kg) in a week.

Watchful Waiting

If you think your child or you may have nephrotic syndrome, or if your child or you have the condition and the symptoms are getting worse, don't wait to get treatment. Call your doctor right away.

Who To See

Health professionals who can diagnose nephrotic syndrome include:

Nephrotic syndrome is usually treated by a nephrologist or a pediatric nephrologist, depending on the age of the person affected.

To prepare for your appointment, see the topic Making the Most of Your Appointment.

Exams and Tests

In addition to a medical history and physical exam, other tests to diagnose nephrotic syndrome include:

  • A 24-hour urine collection, which measures the total amount of protein in the urine collected over 24 hours. You will be diagnosed with nephrotic syndrome if you have more than 3 grams of protein in your urine.
  • Blood test for albumin. Lower levels of albumin in the blood can cause fluid to collect in the ankles, lungs, or abdomen.
  • Creatinine and creatinine clearance. Results of these tests give information on how well your kidneys are working.
  • Blood profile tests to measure the amount of protein, cholesterol, and sugar (glucose) in the blood.
  • Kidney ultrasound to look at the kidneys. This exam can rule out other causes of your symptoms.

You may need other tests before treatment for nephrotic syndrome begins, including:

In adults, testing usually includes:

Unless nephrotic syndrome is clearly caused by diabetes, a kidney biopsy is usually done to find the cause. Children do not usually have a biopsy.

Treatment Overview

Treatment for nephrotic syndrome depends on the cause and the age of the person who has the condition. Medicines, changes in diet, and care for other conditions, such as diabetes or high blood pressure, are all possible treatments for this syndrome. These treatments may reverse, slow or prevent further kidney damage.

Most children who have nephrotic syndrome do well with treatment and have a normal life expectancy. Children older than age 12 at the time of diagnosis and adults who also have diabetes or high blood pressure do not respond as well to treatment as do children younger than 12.

Doctors define complete recovery as living without symptoms or treatment for more than 2 years.

Initial treatment

Treatment of nephrotic syndrome depends on the cause of the disease and may include:

  • Corticosteroids, such as prednisone or prednisolone, to reduce swelling.
  • Diuretics, such as bumetanide (Bumex) or furosemide (Lasix), to reduce fluid buildup in the body (edema) and help with reducing sodium, potassium, and water. Fluid reduction should occur slowly to avoid further kidney damage and low blood pressure.
  • Medicines, such as angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs), to reduce the amount of protein lost in the urine, lower blood pressure, and slow the progress of the disease.
  • In rare cases, salt-free albumin given through a vein (IV). Albumin helps remove extra fluid from the tissues.

You may need emotional support during treatment for nephrotic syndrome. If you or your child has nephrotic syndrome and you are having a hard time handling treatment or the severity of your child's condition, talking with a doctor or seeking counseling may help.

First treatments can last from 6 to 15 weeks, often longer in adults. Depending on how severe your symptoms are or whether they return, ongoing treatment may be necessary for months to years, or even for the rest of your life.

Ongoing treatment

Ongoing treatment for nephrotic syndrome and complications of the disease include:

  • Daily or alternate-day prednisone, if nephrotic syndrome returns.
  • Cyclophosphamide, cyclosporine, or mycophenolate mofetil, when treatment with corticosteroids is not successful.
  • Steps to lower blood pressure, including medicine, a healthier diet, and exercise. Untreated high blood pressure increases your risk for stroke or heart attack. For more information, see the topics High Blood Pressure (Hypertension), Coronary Artery Disease, and Stroke.
  • Changes in diet to replace nutrients lost through the urine, reduce fluid buildup in the body, and reduce the risk of complications. Some doctors prescribe a diet that limits protein, salt (sodium), and fats but is high in carbohydrates. The amount of protein allowed may vary, depending on your condition.
  • Anticoagulants, such as warfarin (Coumadin) or heparin, to treat blood clots if they form.
  • Early treatment of infections with antibiotics.
  • Vaccinations with a pneumococcal vaccine(What is a PDF document?) , chickenpox (varicella) vaccine, and a yearly flu shot. Vaccination is not recommended until nephrotic syndrome has responded to treatment with corticosteroids.
  • Calcium and vitamin D supplements to protect your bones and help prevent osteoporosis during long-term corticosteroid treatment (for example, prednisone).

You can help slow the progression of kidney damage caused by nephrotic syndrome by:

  • Keeping blood pressure at less than 125/75 mm Hg with medicine, diet, and exercise. For more information, see the topic High Blood Pressure (Hypertension).
  • Keeping strict blood glucose control if you have diabetes. For more information, see the topics Type 1 Diabetes and Type 2 Diabetes.
  • Maintaining healthy levels of fats (lipids), such as cholesterol and triglycerides. For more information, see the topic High Cholesterol.
  • Not smoking or using other tobacco products. For more information, see the topic Quitting Smoking.
  • Avoiding medicines that may harm the kidneys.
  • Avoiding X-ray tests that use contrast material.
  • Preventing coronary artery disease. Lifestyle changes such as eating a low-fat diet, quitting smoking, and getting regular exercise can help reduce your overall risk of developing heart disease and stroke. For more information, see the topic Coronary Artery Disease.
  • Weighing yourself daily so that you are aware of any rapid weight gain.

You may need emotional support during treatment for nephrotic syndrome. If you or your child has nephrotic syndrome and you are having a hard time handling treatment or the severity of your child's condition, talking with a doctor or seeking counseling may help.

Treatment if the condition gets worse

Sometimes treatment for nephrotic syndrome is unsuccessful. If this occurs, you may develop chronic kidney disease. Your doctor may recommend that you begin hemodialysis, peritoneal dialysis, or consider a kidney transplant. For more information, see the topic Chronic Kidney Disease.

Clinical trials are ongoing to test more effective medicines for the treatment of steroid-resistant (relapsing) nephrotic syndrome. If treatment has not successfully controlled your nephrotic syndrome, ask your doctor about clinical trials. To take part in a clinical trial, you may need to travel to a large treatment center.

Prevention

Avoiding situations or controlling the other diseases that can contribute to kidney disease can sometimes prevent nephrotic syndrome. The earlier a person changes factors that damage the kidneys, the better. You can help prevent kidney damage by:

If you were diagnosed with nephrotic syndrome in the past:

  • Avoid dehydration. For more information, see the topic Dehydration.
    • Promptly treat illnesses that cause dehydration, such as diarrhea, vomiting, or fever.
    • Prevent dehydration during hot weather and when you exercise. Drink 8 to 10 glasses of fluids (water or rehydration drinks) each day. Drink extra water before, during, and after exercise. Take a container of water or sports drink with you when you exercise. And try to drink at least every 15 to 20 minutes. Use a sports drink if you will be exercising for longer than 1 hour.
    • Avoid caffeine drinks, such as coffee and colas. They increase urine output, which increases dehydration.
    • Avoid alcoholic beverages. They increase urine output, which increases dehydration.
    • Do not take salt tablets. Most people get plenty of salt in their diets. Use a sports drink if you are worried about replacing minerals lost through sweating. Do not use a sports drink if you have heart failure unless you are instructed to do so by your doctor.
    • Stop working outdoors or exercising at the first sign of dizziness, lightheadedness, or fatigue.
    • Wear one layer of lightweight, light-colored clothing when you are working or exercising outdoors. Replace sweaty clothing with dry clothing as soon as you can.
  • Avoid medicines that may harm the kidney.
  • Avoid X-ray tests that use contrast material.
  • Prevent heart disease. Lifestyle changes—eating a low-fat diet, quitting smoking, and getting regular exercise—can help reduce your overall risk of developing heart disease and stroke. For more information, see the topic Coronary Artery Disease.

Home Treatment

People with nephrotic syndrome need to see a doctor to have their kidney function checked regularly. Children usually need to see a doctor more often than adults do.

Take all of your medicines, even after you begin to feel better. If you have side effects, contact your doctor before stopping a medicine.

If you are taking medicines for another health problem, you may need blood testing to see if you are getting the correct amount of medicine. Make sure your doctor knows about all the prescription and nonprescription medicines that you are taking.

You may help reduce symptoms of nephrotic syndrome and prevent other health problems by:

  • Reducing the amount of salt you use, which can reduce the amount of water your body retains.
  • Choosing foods low in fat, which can reduce the amount of cholesterol and triglycerides in your blood.
  • Following your doctor's advice for the amount of protein you need in your diet. It may be a good idea to not eat a high-protein diet. But eating a low-protein diet is not usually recommended, because you may not get enough nutrition.
  • Weighing yourself each day to check for an increase in your weight, which may be a sign that you are retaining fluid. Call your doctor if you notice you are gaining weight or if you develop other problems, such as difficulty breathing.
  • Practicing good skin care to prevent additional skin problems, such as skin infections.

You may need emotional support during treatment for nephrotic syndrome. If you or your child has nephrotic syndrome and you are having a hard time handling treatment or the severity of your child's condition, talking with a doctor or seeking counseling may help.

Medications

Nephrotic syndrome is usually successfully treated with a combination of medicines.

Medication Choices

Medicines to treat nephrotic syndrome include:

  • Corticosteroids, such as prednisone or prednisolone, to reduce swelling.
  • Diuretics, such as bumetanide or furosemide, to help maintain fluid balance.
  • Cyclophosphamide, cyclosporine, or mycophenolate mofetil, when treatment with corticosteroids is not successful.
  • Albumin, to restore blood volume.

Most children are successfully treated with corticosteroids, though relapses are common.1

Clinical trials are ongoing to test more effective medicines for the treatment of steroid-resistant (relapsing) nephrotic syndrome. If treatment has not successfully controlled your nephrotic syndrome, ask your doctor about information on clinical trials. To take part in a clinical trial, you may need to travel to a large treatment center.

What To Think About

Most children who have nephrotic syndrome do well with treatment and have a normal life expectancy. Children older than age 12 at the time of diagnosis and adults who also have diabetes or high blood pressure do not respond as well to treatment as do children younger than 12.

Surgery

Surgery is not used to treat nephrotic syndrome. But if treatment is not controlling your condition and you have developed chronic kidney disease, your doctor may recommend a kidney transplant.

Other Treatment

There are no other treatments for nephrotic syndrome.

Other Places To Get Help

Organizations

American Kidney Fund
6110 Executive Boulevard
Suite 1010
Rockville, MD  20852
Phone: 1-800-638-8299
1-866-300-2900 helpline
E-mail: helpline@kidneyfund.org
Web Address: www.kidneyfund.org
 

The American Kidney Fund is a national voluntary health organization dedicated to improving the daily lives of people who have chronic kidney disease. Its goal as a patient aid program is to relieve the financial burden associated with chronic kidney failure. Also, the organization's information service provides information about how to prevent and treat kidney disease and about the great need for organ donors.


National Kidney and Urologic Diseases Information Clearinghouse
3 Information Way
Bethesda, MD  20892-3580
Phone: 1-800-891-5390
Fax: (703) 738-4929
TDD: 1-866-569-1162 toll-free
E-mail: nkudic@info.niddk.nih.gov
Web Address: http://kidney.niddk.nih.gov
 

The National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC), a federal agency, is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). NIDDK is part of the National Institutes of Health under the U.S. Department of Health and Human Services. The clearinghouse provides information about diseases of the kidneys and urologic system to people with kidney and urologic disorders and to their families, to health professionals, and to the public. NKUDIC answers inquiries; develops, reviews, and distributes publications; and works closely with professional and patient organizations and government agencies to coordinate resources about kidney and urologic diseases.


National Kidney Foundation
30 East 33rd Street
New York, NY  10016
Phone: 1-800-622-9010
(212) 889-2210
Fax: (212) 689-9261
Web Address: www.kidney.org
 

The National Kidney Foundation works to prevent kidney and urinary tract diseases and help people affected by these conditions. Its Web site has a lot of information about adult and child conditions. The site has interactive tools, donor information, recipes for kidney disease patients, and message boards for many kidney topics. Free materials, such as brochures and newsletters, are available.


References

Citations

  1. Eddy AA, Symons JM (2003). Nephrotic syndrome in childhood. Lancet, 362(9384): 629–639.

Other Works Consulted

  • Nachman PH, et al. (2008). Primary glomerular disease. In BM Brenner, SA Levine, eds., Brenner and Rector's The Kidney, 8th ed., vol. 1, pp. 987–1066. Philadelphia: Saunders Elsevier.

Credits

Author Monica Rhodes
Editor Kathleen M. Ariss, MS
Associate Editor Pat Truman, MATC
Primary Medical Reviewer Anne C. Poinier, MD - Internal Medicine
Specialist Medical Reviewer Mitchell H. Rosner, MD - Nephrology
Last Updated June 5, 2009

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