Topic Overview
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. You 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
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Being diagnosed:
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Living with nephrotic syndrome:
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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.
- Infections, such as
HIV, hepatitis B, or hepatitis C.
- Cancer
- 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 things 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 have complications from the other condition also.
Most children who have nephrotic syndrome do well with treatment and have
a normal life expectancy.
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.
You also have an increased risk of developing nephrotic
syndrome if you have a disease that results in an
impaired immune system, such as
HIV 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:
- Chest pain.
- Severe shortness of breath or
difficulty breathing.
- Sudden and severe belly pain or flank pain. Flank pain is felt just below the rib cage and above the waist on one or both sides of the back.
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. These include:
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.
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 to reduce fluid buildup in the body (edema) and to 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.
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 needed 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,
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 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, it may help to talk with a doctor or seek
counseling.
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
the things 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.
- 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 heart disease and
stroke. For more information, see the topic
Coronary Artery Disease.
Home Treatment
People who have
nephrotic syndrome need to see a doctor regularly to have their
kidney function checked. 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.
Healthy Eating: Eating Less Sodium
- 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.
- 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.
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.
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 |
| Email: |
helpline@kidneyfund.org |
| Web Address: |
www.kidneyfund.org |
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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.
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| 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 |
| Email: |
nkudic@info.niddk.nih.gov |
| Web Address: |
http://kidney.niddk.nih.gov |
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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 groups and government agencies to
coordinate resources about kidney and urologic diseases.
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| National Kidney Foundation |
| 30 East 33rd Street |
| New York, NY 10016 |
| Phone: |
1-800-622-9010 |
| Phone: |
(212) 889-2210 |
| Fax: |
(212) 689-9261 |
| Web Address: |
www.kidney.org |
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|
The National Kidney Foundation works to prevent kidney
and urinary tract diseases and help people affected by these conditions. Its
website 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.
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References
Citations
- American Academy of Pediatrics (2009). Management of childhood onset nephrotic syndrome. Pediatrics, 124(2): 747–757. Available online: http://www.pediatrics.org/cgi/content/full/124/2/747.
Other Works Consulted
- Kodner C (2009). Nephrotic syndrome in adults: Diagnosis and management. American Family Physician, 80(10): 1129–1134.
- Lewis JB, Neilson EG (2008). Glomerular diseases. In
AS Fauci et al., eds., Harrison's Principles of Internal Medicine, 17th ed., vol. 2, pp. 1782–1797. New York:
McGraw-Hill.
- 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.
- Praga M et al. (2011). Primary glomerular diseases In ET Bope et al., eds., Conn's Current Therapy 2011, pp. 714–719. Philadelphia: Saunders.
- Vincenti FG, Amend WJC (2008). Nephrotic syndrome
section of Diagnosis of medical renal diseases. In EA Tanagho, JW McAninch,
eds., Smith's General Urology, 17th ed., pp. 524–526.
New York: McGraw-Hill.
- Watnik S, et al. (2010). Nephrotic syndrome section of Kidney disease. In SJ McPhee, MA Papadakis, eds., Current Medical Diagnosis and Treatment 2010, 49th ed., pp. 838–840. New York: McGraw-Hill.
Credits
| By |
Healthwise Staff |
| Primary Medical Reviewer |
Anne C. Poinier, MD - Internal Medicine |
| Specialist Medical Reviewer |
Tushar J. Vachharajani, MD, FASN, FACP - Nephrology |
| Last Revised |
May 7, 2012 |
