What Is Sarcoidosis?
Sarcoidosis (sar-koy-DO-sis) is a disease of unknown
cause that leads to inflammation. It can affect various organs in the body.
Normally, your immune system defends your body
against foreign or harmful substances. For example, it sends special cells to
protect organs that are in danger.
These cells release chemicals that recruit other
cells to isolate and destroy the harmful substance. Inflammation occurs during
this process. Once the harmful substance is destroyed, the cells and the
inflammation go away.
In people who have sarcoidosis, the inflammation
doesn't go away. Instead, some of the immune system cells cluster to form lumps
called granulomas (gran-yu-LO-mas) in various organs in your body.
Overview
Sarcoidosis can affect any organ in your body.
However, it's more likely to occur in some organs than in others. The disease
usually starts in the lungs, skin, and/or lymph nodes (especially the lymph
nodes in your chest).
The disease also often affects the eyes and the
liver. Although less common, sarcoidosis can affect the heart and brain,
leading to serious complications.
If many granulomas form in an organ, they can affect
how the organ works. This can cause signs and symptoms. Signs and symptoms vary
depending on which organs are affected. Many people who have sarcoidosis have
no symptoms or mild symptoms.
Lofgren's syndrome is a classic set of signs and
symptoms that is typical in some people who have sarcoidosis. Lofgren's
syndrome may cause fever, enlarged lymph nodes, arthritis (usually in the
ankles), and/or erythema nodosum (er-i-THE-ma no-DO-sum).
Erythema nodosum is a rash of red or reddish-purple
bumps on your ankles and shins. The rash may be warm and tender to the
touch.
Treatment for sarcoidosis also varies depending on
which organs are affected. Your doctor may prescribe topical treatments and/or
medicines to treat the disease. Not everyone who has sarcoidosis needs
treatment.
Outlook
The outcome of sarcoidosis varies. Many people
recover from the disease with few or no long-term problems.
More than half of the people who have sarcoidosis
have remission within 3 years of diagnosis. Remission means the
disease isn't active, but it can return.
Two-thirds of people who have the disease have
remission within 10 years of diagnosis. People who have Lofgren's syndrome
usually have remission. Relapse (return of the disease) 1 or more years after
remission occurs in less than 5 percent of patients.
Sarcoidosis leads to organ damage in about one-third
of the people diagnosed with the disease. Damage may occur over many years and
involve more than one organ. Rarely, sarcoidosis can be fatal. Death usually is
the result of complications with the lungs, heart, or brain.
Poor outcomes are more likely in people who have
advanced disease and show little improvement from treatment.
Certain people are at higher risk for poor outcomes
from chronic (long-term) sarcoidosis. This includes people who have lung
scarring, heart or brain complications, or lupus pernio (LU-pus PAR-ne-o).
Lupus pernio is a serious skin condition that sarcoidosis may cause.
Research is ongoing for new and better treatments
for sarcoidosis.
What Causes Sarcoidosis?
The cause of sarcoidosis isn't known. More than one
factor may play a role in causing the disease.
Some researchers think that sarcoidosis develops
when your immune system responds to a trigger, such as bacteria, viruses, dust,
or chemicals.
Normally, your immune system defends your body
against foreign or harmful substances. For example, it sends special cells to
protect organs that are in danger.
These cells release chemicals that recruit other
cells to isolate and destroy the harmful substance. Inflammation occurs during
this process. Once the harmful substance is destroyed, the cells and the
inflammation go away.
In people who have sarcoidosis, the inflammation
doesn't go away. Instead, some of the immune system cells cluster to form lumps
called granulomas in various organs in your body.
Genetics also may play a role in sarcoidosis.
Researchers believe that sarcoidosis occurs if:
- You have a certain gene (or certain genes) that
raise your risk for the disease
- And
- You're exposed to something that triggers your
immune system
Triggers may vary depending on your genetic makeup.
Certain genes may influence which organs are affected and how severe your
symptoms are.
Researchers continue to try to pinpoint the genes
that are linked to sarcoidosis.
Who Is At Risk for Sarcoidosis?
Sarcoidosis affects people of all ages and races.
However, it's more common among African Americans and Northern Europeans. In
the United States, the disease affects African Americans somewhat more often
and more severely than Whites.
Studies have shown that sarcoidosis tends to vary in
different ethnic groups. For example, eye problems due to the disease are more
common in Japanese people.
Lofgren's syndrome, a type of sarcoidosis, is more
common in people of European descent. Lofgren's syndrome may involve fever,
enlarged lymph nodes, arthritis (usually in the ankles), and/or erythema
nodosum. Erythema nodosum is a rash of red or reddish-purple bumps on your
ankles and shins. The rash may be warm and tender to the touch.
Sarcoidosis is somewhat more common in women than in
men. The disease usually develops between the ages of 20 and 50.
People who have certain jobs also may be at higher
risk for sarcoidosis. Examples include:
- Health care workers
- Elementary and secondary school teachers
- People whose jobs expose them to agricultural
dust, insecticides, pesticides, or mold
- Suppliers of building materials, hardware, or
gardening materials
- Firefighters
People who have a family history of sarcoidosis also
are at higher risk for the disease.
What Are the Signs and Symptoms of
Sarcoidosis?
Many people who have sarcoidosis have no symptoms or
mild symptoms. Often, the disease is found during a
chest
x ray done for another reason (for example, to diagnose
pneumonia).
The signs and symptoms of sarcoidosis vary depending
on which organs are affected. Signs and symptoms also may vary depending on
your gender, age, and ethnic background. (For more information, see
"Who
Is At Risk for Sarcoidosis?")
Common Signs and Symptoms
In both adults and children, the disease most often
affects the lungs. If granulomas (inflamed lumps) form in your lungs, you may
wheeze, cough, feel short of breath, or have chest pain. Or, you may have no
symptoms at all.
Some people who have sarcoidosis feel very fatigued
(tired), uneasy, or depressed. Night sweats and weight loss are common symptoms
of the disease.
Common signs and symptoms in children are fatigue,
loss of appetite, weight loss, bone and joint pain, and
anemia.
Children who are younger than 4 years old may have a distinct form of
sarcoidosis. It may cause enlarged lymph nodes in the chest (which can be seen
on a chest x ray), skin lesions, and eye swelling or redness.
Other Signs and Symptoms
Sarcoidosis may affect your lymph nodes. The disease
can cause enlarged lymph nodes that feel tender. Sarcoidosis usually affects
the lymph nodes in your neck and chest. However, the disease also may affect
the lymph nodes under your chin, in your armpits, or in your groin.
Sarcoidosis can cause lumps, ulcers, or areas of
discolored skin. They may itch, but they don't hurt. These signs tend to appear
on your back, arms, legs, and scalp. Sometimes they appear near your nose or
eyes. These signs usually last a long time.
Sarcoidosis may cause a more serious skin condition
called lupus pernio. Disfiguring skin sores may affect your nose, nasal
passages, cheeks, ears, eyelids, and fingers. These sores tend to be ongoing.
They can return after treatment is over.
Sarcoidosis also can cause eye problems. If you have
sarcoidosis, it's important to have an annual eye exam. If you have changes in
your vision and can't see as clearly or can't see color, call 911
or have someone drive you to the emergency room.
You should call your doctor if you have any new eye
symptoms, such as burning, itching, tearing, pain, or sensitivity to light.
Signs and symptoms of sarcoidosis also may include
an enlarged liver, spleen, or salivary glands.
Although less common, sarcoidosis can affect the
heart and brain. This can cause a number of symptoms, such as abnormal
heartbeats, shortness of breath, headaches, and vision problems. If sarcoidosis
affects the heart or brain, serious complications can occur.
Lofgren's Syndrome
Lofgren's syndrome is a classic set of signs and
symptoms that appear in some people when they first develop sarcoidosis. Signs
and symptoms may include:
- Fever. This symptom only occurs in some
people.
- Enlarged lymph nodes (which can be seen on a
chest x ray).
- Arthritis, usually in the ankles. This symptom is
more common in men.
- Erythema nodosum. This is a rash of red or
reddish-purple bumps on your ankles and shins. The rash may be warm and tender
to the touch. This symptom is more common in women.
Sarcoidosis Signs and
Symptoms
The illustration shows the major
signs and symptoms of sarcoidosis and the organs involved.
How Is Sarcoidosis Diagnosed?
Your doctor will diagnose sarcoidosis based on your
medical history, a physical exam, and the results from tests. He or she will
look for granulomas (inflamed lumps) in your organs. Your doctor also will try
to rule out other causes of your symptoms.
Medical History
Your doctor may ask you detailed questions about
your medical history. For example, he or she may ask whether you have a family
history of sarcoidosis. Your doctor also may ask whether you've had any jobs
that may have raised your risk for the disease.
Your doctor also may ask whether you've ever been
exposed to inhaled beryllium metal. This type of metal is used to make
aircrafts and weapons. Your doctor also may want to know whether you've had
contact with organic dust from birds or hay.
Exposure to these substances can cause inflamed
lumps in your lungs that look like the granulomas from sarcoidosis. However,
these lumps are signs of other conditions.
Physical Exam
Your doctor will examine you for signs and symptoms
of sarcoidosis. Signs and symptoms may include red bumps on your skin; swollen
lymph nodes; an enlarged liver, spleen, or salivary glands; or redness in your
eyes. He or she will check for other causes of your symptoms.
Your doctor also may listen to your lungs and heart.
Abnormal breathing and heartbeat sounds may be a sign that sarcoidosis is
affecting your lungs or heart.
Diagnostic Tests
You may have tests to confirm a diagnosis and to
find out how the disease is affecting you. Tests include
chest
x ray,
lung
function tests, biopsy, and other tests to assess organ damage.
Chest X Ray
A chest x ray is a painless test that creates
pictures of the structures inside your chest, such as your heart and lungs. The
test may show granulomas or enlarged lymph nodes in your chest. About 95
percent of people who have sarcoidosis have an abnormal chest x ray.
Lung Function Tests
Lung function tests measure the size of your lungs,
how much air you can breathe in and out, how fast you can breathe air out, and
how well your lungs deliver oxygen to your blood. These tests may be used to
find out whether sarcoidosis is affecting your lungs.
Biopsy
Your doctor may do a biopsy to confirm a diagnosis
or rule out other causes of your symptoms. A biopsy involves taking a small
sample of tissue from one of your affected organs.
Usually, doctors try to biopsy the organs that are
easiest to access. Examples include the skin, tear glands, or the lymph nodes
that are just under the skin.
If this isn't possible, your doctor may use a
positron emission tomography (PET) scan to pinpoint areas for biopsy. For this
test, a small amount of a radioactive substance is injected into a vein,
usually in your arm.
The substance, which releases energy, travels
through the blood and collects in organs or tissues. Special cameras detect the
energy and convert it into three-dimensional pictures.
If lung function tests or a chest x ray shows signs
of sarcoidosis in your lungs, your doctor may do a bronchoscopy
(bron-KOS-ko-pee) to get a small sample of lung tissue.
During this procedure, a thin, flexible tube is
passed through your nose (or sometimes your mouth), down your throat, and into
the airways to reach your lung tissue. (For more information, see
"What
To Expect During Bronchoscopy.")
Other Tests To Assess Organ Damage
You also may have other tests to assess organ damage
and find out whether you need treatment. For example, your doctor may recommend
blood
tests and/or an
EKG
(electrocardiogram).
Everyone who is diagnosed with sarcoidosis should
see an ophthalmologist (eye specialist) for eye tests, even if they don't have
eye symptoms. This is important because eye damage can occur without
symptoms.
How Is Sarcoidosis Treated?
Not everyone who has sarcoidosis needs treatment. In
some cases, the disease goes away on its own. Whether you need treatment and
what type of treatment you need depend on your signs and symptoms, which organs
are affected, and whether those organs are working well.
If the disease affects certain organs, such as your
eyes, heart, or brain, you'll need treatment even if you don't have any
symptoms.
In either casewhether you have symptoms or
notyou should see your doctor for ongoing care. He or she will want to
check to make sure that the disease isn't damaging your organs. For example,
you may need
lung
function tests to make sure that your lungs continue to work well.
If the disease isn't worsening, your doctor may
watch you closely to see whether the disease goes away on its own. If the
disease does start to get worse, your doctor can prescribe treatment.
The goals of treatment are to:
- Relieve symptoms
- Improve organ function
- Control inflammation and reduce the size of
granulomas (inflamed lumps)
- Prevent pulmonary fibrosis (lung scarring) if
your lungs are affected
Your doctor may prescribe topical treatments and/or
medicines to treat the disease.
Medicines
Prednisone
Prednisone, a type of steroid, is the main treatment
for sarcoidosis. This medicine reduces inflammation. In most people, prednisone
relieves symptoms within a couple of months.
Although most people need to take prednisone for 12
months or longer, your doctor may lower the dose within a few months after you
start the medicine.
Long-term use of prednisone, especially at high
doses, can cause serious side effects. Work with your doctor to decide whether
the benefits of this medicine outweigh the risks. If your doctor prescribes
this treatment, he or she will find the lowest dose that controls your
disease.
When you stop taking prednisone, you should cut back
slowly (as your doctor advises). This will help prevent flareups of
sarcoidosis. Cutting back slowly also allows your body to adjust to not having
the medicine.
If a relapse or flareup occurs after you stop taking
prednisone, you may need a second round of treatment. If you remain stable for
more than 1 year after stopping this treatment, the risk of relapse is low.
Other Medicines
Other medicines, besides prednisone, also are used
to treat sarcoidosis. Examples include:
- Hydroxychloroquine. This medicine works best for
treating sarcoidosis that affects the skin or brain. Your doctor also may
prescribe this medicine if you have a high level of calcium in your blood due
to sarcoidosis.
- Methotrexate. This medicine works best for
treating sarcoidosis that affects your lungs, eyes, skin, or joints.
Your doctor may prescribe these medicines if your
sarcoidosis worsens while you're taking prednisone or if you can't handle
prednisone's side effects.
If you have Lofgren's syndrome with pain or fever,
your doctor may prescribe nonsteroidal anti-inflammatory drugs (NSAIDs), such
as ibuprofen.
If you're wheezing and coughing, you may need
inhaled medicine to help open your airways. You take inhaled medicine using an
inhaler. This device allows the medicine to go right to your lungs.
Ongoing Research
Researchers continue to look for new and better
treatments for sarcoidosis. They're currently studying treatments aimed at the
immune system. Researchers also are studying antibiotics as a possible
treatment for sarcoidosis that affects the skin.
Living With Sarcoidosis
Sarcoidosis has no cure. However, you can take steps
to manage the disease. Get ongoing care and follow a healthy lifestyle. Talk to
your doctor if youre pregnant or planning a pregnancy.
Ongoing Care
Getting ongoing care is important, even if you don't
take medicine for your sarcoidosis. New symptoms can occur at any time. Also,
the disease can slowly worsen without your noticing.
How often you need to see your doctor will depend on
how severe your symptoms are, which organs are affected, what treatments you're
using, and whether you have any side effects from treatments. Even if you
dont have symptoms, you should see your doctor for ongoing care.
Your doctor may recommend routine tests, such as
lung
function tests and eye exams. He or she will want to check to make sure
that the disease isnt damaging your organs.
Discuss with your doctor how often you need to have
followup visits. You may have some followup visits with your primary care
doctor and others with one or more specialists.
Lifestyle Changes
Making lifestyle changes can help you manage your
health. For example, follow a healthy diet and be as physically active as you
can. A healthy diet includes a variety of fruits, vegetables, and whole
grains.
It also includes lean meats, poultry, fish, beans,
and fat-free or low-fat milk or milk products. A healthy diet is low in
saturated fat, trans fat, cholesterol, sodium (salt), and added
sugar.
For more information on following a healthy diet,
see the National Heart, Lung, and Blood Institute's
Aim for a
Healthy Weight Web site,
"Your
Guide to a Healthy Heart," and
"Your
Guide to Lowering Your Blood Pressure With DASH." All of these resources
include general information about healthy eating.
If you smoke, quit. Talk to your doctor about
program and products that can help you quit. Also, try to avoid other lung
irritants, such as dust, chemicals, and secondhand smoke.
Emotional Issues
Living with a chronic disease may cause fear,
anxiety, depression, and stress. Its important to talk about how you feel
with your health care team. Talking to a professional counselor also can help.
If youre feeling very depressed, your health care team or counselor may
prescribe medicines to make you feel better.
Joining a patient support group may help you adjust
to living with sarcoidosis. You can see how other people who have the disease
have coped with it. Talk to your doctor about local support groups or check
with an area medical center.
Support from family and friends also can help
relieve stress and anxiety. Let your loved ones know how you feel and what they
can do to help you.
Pregnancy
Many women who have sarcoidosis give birth to
healthy babies. Women who have severe sarcoidosis, especially if theyre
older, may have trouble becoming pregnant. In some cases, sarcoidosis may get
worse after the baby is delivered.
If you have sarcoidosis and are pregnant or planning
a pregnancy, talk to your doctor about the risks. Also, if you become pregnant,
its important to get good prenatal care and regular sarcoidosis checkups
during and after pregnancy.
Some sarcoidosis medicines are considered safe to
use during pregnancy; others are not recommended.
Key Points
- Sarcoidosis is a disease of unknown cause that
leads to inflammation. It can affect various organs in the body.
- Sarcoidosis is more likely to occur in some
organs than in others. The disease usually starts in the lungs, skin, and/or
lymph nodes (especially the lymph nodes in your chest). The disease also often
affects the eyes and the liver.
- In people who have sarcoidosis, immune system
cells cause inflammation and cluster to form lumps called granulomas.
- If many granulomas form in an organ, they can
affect how the organ works. This can cause signs and symptoms. Signs and
symptoms vary depending on which organs are affected. Many people who have
sarcoidosis have no symptoms or mild symptoms.
- Some researchers think that sarcoidosis develops
when your immune system responds to a trigger, such as bacteria, viruses, dust,
or chemicals. Genetics also may play a role in sarcoidosis.
- Sarcoidosis affects people of all ages and races.
In the United States, the disease affects African Americans somewhat more often
and more severely than Whites. The disease also is slightly more common in
women than in men. It usually develops between the ages of 20 and 50.
- In both adults and children, the disease most
often affects the lungs. If granulomas form in your lungs, you may wheeze,
cough, feel short of breath, or have chest pain. Some people who have
sarcoidosis feel very fatigued (tired), uneasy, or depressed. Night sweats and
weight loss are common symptoms of the disease.
- Lofgrens syndrome is a classic set of signs
and symptoms that appear in some people when they first develop sarcoidosis.
Signs and symptoms may include fever, enlarged lymph nodes, arthritis, and
erythema nodosum (a rash of red or reddish-purple bumps on your ankles).
- Your doctor will diagnose sarcoidosis based on
your medical history, a physical exam, and the results from tests. He or she
will look for granulomas (inflamed lumps) in your organs. Your doctor also will
try to rule out other causes of your symptoms.
- Whether you need treatment and what type of
treatment you need depend on your signs and symptoms, which organs are
affected, and whether those organs are working well.
- Your doctor may prescribe topical treatments
and/or medicines to treat the disease. Not everyone who has sarcoidosis needs
treatment. In some cases, the disease goes away on its own.
- If the disease affects certain organs, such as
your eyes, heart, or brain, you may need treatment even if you dont have
any symptoms.
- Sarcoidosis has no cure. However, you can take
steps to manage the disease. Get ongoing care and follow a healthy lifestyle.
Talk to your doctor if youre pregnant or planning a pregnancy.
- Getting ongoing care is important, even if you
dont take medicine for your sarcoidosis. New symptoms can occur at any
time. Also, your condition can slowly worsen without your noticing.
- The outcome of sarcoidosis varies. Many people
recover from the disease with few or no long-term problems.
- Rarely, sarcoidosis can be fatal. Death usually
is the result of complications with the lungs, heart, or brain. Poor outcomes
are more likely in people who have advanced disease and show little improvement
from treatment.
Links to Other Information About Sarcoidosis
Non-NHLBI Resources
Clinical Trials
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