Treatment Guide
Sarcoidosis
CHOOSING YOUR CARE
Since sarcoidosis is a multi-system disorder that can
affect many different organs, patients often seek treatment
from doctors who specialize in treating the particular
organ or system that is being affected.
Cleveland Clinic’s Sarcoidosis Center of Excellence has
been developed to improve the care of patients and to
teach individuals with sarcoidosis how to live and better
cope with their disease.
At the Sarcoidosis Center, we make sure patients
quickly see the best doctors for their case by using a
multidisciplinary approach. Our team of doctors, who
have expertise and an interest in sarcoidosis, includes
specialists in pulmonology, cardiology, electrophysiology,
neurology, ophthalmology, dermatology and rheumatology.
Together, we can help most people with sarcoidosis lead
normal and active lives.
Cleveland clinic sarcoidosis Center of Excellence
Cleveland Clinic’s Respiratory Institute has
a long history of excellence and innovation,
and is ranked the #3 pulmonology program
in the nation by U.S.News & World Report.
Cleveland Clinic also is consistently ranked
among America’s Top 4 hospitals.
Using this guide
Please use this guide as a resource as you
learn about sarcoidosis causes, diagnoses
and treatment options. As a patient, you
have the right to ask questions and to seek
a second option.
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What is sarcoidosis?
How Sarcoidosis
Got its Name
Sarcoidosis was first identified
more than 100 years ago by two
dermatologists – Dr. Jonathan
Hutchinson in England and
Dr. Caesar Boeck in Norway.
The disease was first named
both Hutchinson’s disease and
Boeck’s disease.
Dr. Boeck later renamed the disease sarcoidosis from the Greek
words “sark” and “oid,” meaning flesh-like – a term describing
the skin eruptions that are
frequently seen in patients with
the disease.
Sarcoidosis is an inflammatory disease that affects one or more organs, but
most commonly affects the lungs and lymph glands. As a result of the inflammation, abnormal lumps or nodules (called granulomas) form in one or more organs
of the body. These granulomas may change the normal structure and possibly
the function of the affected organ(s).
Who tends to get sarcoidosis?
Although it occurs throughout the world and can affect individuals of all ages,
races and both genders, sarcoidosis most commonly strikes adults between 20
and 40 years of age. It also is more common in blacks and in people of Scandinavian, German, Irish and Puerto Rican descent. No one knows why. White
women are just as likely as white men to get sarcoidosis; however, black
women get sarcoidosis two times more often than black men.
Worldwide, the disease occurs in up to four in 10,000 people. In the United
States, sarcoidosis is diagnosed in about one to two whites per 10,000 and
in three to four blacks per every 10,000. Since sarcoidosis mimics many other
diseases, the number of people who actually have sarcoidosis is thought to
be considerably higher than the number reported.
The overall mortality rate for sarcoidosis ranges from 1 percent to 6 percent,
with most deaths occurring in those with long-standing, progressive disease.
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What causes sarcoidosis?
The exact cause of sarcoidosis is not known. The disease can appear suddenly
and then disappear, or it can develop gradually and produce symptoms that come
and go for a lifetime.
Researchers believe that the disease is caused by an abnormal immune
response. (The body’s defense system does not react as it should to a foreign
substance “intruder.”) In a healthy person, inflammation occurs as the cells of
the body’s immune system come together to fight the intruder at an organ or
tissue site. In a person with sarcoidosis, however, cells that come to fight end
up clumping together into small lumps called granulomas.
It’s still uncertain which foreign substance “triggers” the body’s abnormal
response. Some researchers suggest that fungi, viruses or bacteria are likely
triggers. In fact, cases of sarcoidosis have occurred in groups of people who
had close contact with each other, as well as in recipients of heart, lung and
bone marrow transplants. But, so far, no data have been able to convincingly
and consistently establish this “infectious” connection as the cause of the
disease. However, some types of bacteria have recently emerged as possible
candidates and continue to be closely studied.
Does sarcoidosis run in families?
While the latest research does appear to indicate a genetic susceptibility to
the disease, more research is needed to clearly identify and confirm the genes
involved. However, numerous reports have revealed racial/ethnic and family-line
occurrences, including the following:
• Irish immigrants in London have a three-fold likelihood of developing sarcoidosis
compared with native Londoners.
Symptoms of Sarcoidosis
Most common initial symptoms
Shortness of breath (dyspnea)
Cough that won’t go away
Reddish bumps or patches on the
skin or under the skin
Enlarged lymph glands in the chest
and around the lungs that produce
cough and shortness of breath
Fever, weight loss, fatigue, night
sweats, general feeling of ill health
Other disease characteristics
Red and teary eyes or blurred vision
Swollen and painful joints
Enlarged lymph glands in the neck,
armpits and groin
•N
atives of Martinique living in France have an eight-fold higher chance of
developing the disease compared with the native French population.
Nasal stuffiness and hoarse voice
• Blacks face a four to 17 times greater risk of the disease compared with whites.
Pain in the hands, feet or other
bony areas due to the formation of
cysts (an abnormal sac-like growth)
in bones
•W
ithin individual families, the presence of the disease in a first- or seconddegree relative increases the risk by nearly five-fold.
Still other types of disease clusters have been identified, including seasonal and
occupational clustering. Researchers in Greece, Spain and Japan have reported
a clustering of diagnoses of sarcoidosis in the months of March to May, April
to June, and June to July. In the United States, a higher percentage of cases of
sarcoidosis have been reported in healthcare workers, naval aircraft servicemen
and firefighters.
What are the symptoms of sarcoidosis?
The symptoms of sarcoidosis can vary greatly from individual to individual, and
depend on which tissues and organs are affected. In some people, symptoms may
begin suddenly and/or severely and subside in a short period of time. Others may have
no outward symptoms at all, even though organs are affected. Still others may have
symptoms that appear slowly and subtly, but last or recur over a long time span.
Cleveland clinic sarcoidosis center of excellence
Kidney stone formation
Development of abnormal or missed
heart beats (arrhythmias), inflammation of the covering of the heart
(pericarditis) or heart failure
Nervous system effects include
hearing loss, meningitis, seizures or
psychiatric disorders (for example,
dementia, depression, psychosis)
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How Sarcoidosis Progresses
What happens at the tissue level
At the tissue or cellular level,
sarcoidosis disease progression
can be divided into three phases:
(1) The first change that is seen is
inflammation.
(2) In the second phase, granulomas form. Granulomas are masses
or nodules of chronically inflamed
tissue and are the classic sign of
sarcoidosis. Granulomas are the
body’s attempt to wall off or isolate
organisms and other foreign particles that are difficult for the immune
system to eliminate.
(3) In the third phase, fibrosis (scarring) of tissues or organs occurs. If
scarring is extensive in a vital organ,
sarcoidosis is sometimes fatal.
In some people, the disease advances from one phase to the next in
the tissues of the organ affected. In
others, the different phases of tissue
changes take place within the same
organ at the same time.
In many patients with sarcoidosis,
the granulomas go away on their
own in two to three years without
the patient knowing or doing anything about them. In others, the
granulomas progress to irreversible
fibrosis.
The immune system changes that
allow one person’s disease to progress while another person’s disease
resolves are not well understood
and continue to be investigated.
How does the condition progress?
Although no one can predict how sarcoidosis will progress in an individual patient,
some clues as to disease course can be gained from patient symptoms, findings
from physical and laboratory studies, and patient race. For example, a sudden
onset of general symptoms – such as weight loss, fatigue, fever or just an overall
feeling of ill health – usually means that the course of sarcoidosis will be relatively
short and mild in severity. Symptoms of shortness of breath and some types of
skin involvement mean that sarcoidosis will be more long-lasting and severe.
In whites, the disease often appears suddenly, which usually indicates a more mild
form of the disease that is of short duration. Blacks and Puerto Ricans, on the other
hand, tend to develop the more long-term and severe form of the disease.
In the United States, the lungs are often the most common site of initial symptoms for those who experience a gradual onset of their long-term disease. Lung
symptoms are common in blacks, Puerto Ricans and Scandinavians. Persistent
dry cough, fatigue and shortness of breath are the most common initial lungrelated complaints.
Some patients who experience sudden onset of disease have Lofgren’s syndrome,
a form of sarcoidosis that affects the lymph nodes and is accompanied by a skin
condition that produces red nodules under the skin, as well as fever and arthritis
pain. Patients with Lofgren’s syndrome usually can expect a good outcome; the
disease goes away on its own in 85 percent to 90 percent of people who have it.
How does sarcoidosis
affect various organs?
Beyond the lungs and lymph glands, the body organs or systems affected by
sarcoidosis that are associated with the most noticeable symptoms are the skin,
eyes, musculoskeletal system, nervous system, heart, liver and kidneys, in this
order. Patients can have symptoms related to the specific organ affected, or can
have general symptoms, or can have no symptoms whatsoever (for example, laboratory findings frequently show the liver to be affected by sarcoidosis, yet patients
generally do not report any liver-related symptoms).
An individual patient’s symptoms also can vary according to how long the illness
has been under way, where granulomas are forming, how much tissue has become
affected, and whether granuloma formation is still active or has developed into scar.
Your lungs
The lungs are affected in more than 90 percent of individuals with sarcoidosis.
Even in individuals whose disease primarily affects other organs, the lungs are
usually affected as well. Shortness of breath, cough and chest discomfort are the
most common lung-related symptoms. Patients may be free of chest symptoms
despite an abnormal chest X-ray and biopsy-proven sarcoidosis. Occasionally,
patients have chest pain – which is usually described as a vague tightness of the
chest – but sometimes the pain can be severe and similar to cardiac pain.
Your skin
About 25 percent of patients with sarcoidosis develop at least one skin symptom. Tender, painful, reddish bumps or patches on the skin (erythema nodosum)
– usually on the shins – accompanied by swollen and painful joints are common.
Lupus pernio (a chronic skin condition marked by purple-colored lesions on the
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cheeks, lips, nose and ears) is common in older blacks and West Indian women
with longstanding disease.
Other skin signs in patients with long-term disease include plaques, patches
(with and without color), nodules and hair loss. The range in appearance of
skin-related lesions – from subtle, painless rashes to deep scars – often correlates with the severity of sarcoidosis involving the internal organs. The skin
changes can be visible or under the skin.
Your eyes
About 25 percent of people with sarcoidosis have eye symptoms. Inflammation
of almost any part of the eye can occur – this includes the membranes of the
eyelids, cornea, outer coat of the eyeball (sclera), iris, retina and lens. The most
common eye-related symptom is acute anterior uveitis (inflammation of a layer
of the eye), which results in the rapid onset of blurred vision, teary eyes and
light sensitivity. In long-standing disease, glaucoma, cataracts and blindness can
occur. Dry eyes are very frequent in long-standing sarcoidosis, even when there
is no remaining inflammation; they can be treated with saline drops.
Because some sarcoid-related eye problems do not cause symptoms, it is
important that all patients with sarcoidosis have regularly scheduled appointments with an ophthalmologist.
Your musculoskeletal system
Approximately 10 percent to 15 percent of patients may have bone and muscle symptoms, resulting in arthritis, changes in bone structure, or muscle discomfort and pain.
Your nervous system
Neurologic disease occurs in 5 percent to 10 percent of patients, often without
symptoms in other organs. Symptoms of neurologic involvement include headaches, meningitis, seizures and nerve tissue degeneration or inflammation – which
results in muscle weakness, pain and numbing or tingling sensations in the face,
arms and legs.
A more recently discovered type of neurosarcoidosis is small fiber neuropathy
(SFN). SFN causes a loss of some types of nerve fibers and requires specialized
testing to identify it. Patients with SFN often note burning pain, sensitivity to
touch, palpitations, sweating, flushing, lightheadedness, gastrointestinal difficulties and sexual dysfunction.
Your heart
Heart disease is present in 28 percent of patients with sarcoidosis, although only
about 5 percent of patients report having any heart-related symptoms. Any part of
the heart’s structure may be affected by granuloma formation. The most frequently
diagnosed heart problems include chest pain, cor pulmonale (an enlargement of
the right side of the heart that results from disease in the lungs or its blood vessels), cardiomyopathy (disease of the heart muscle itself), and abnormalities in
the heart’s electrical system, which can result in heart block, dysrhythmias and
sudden death. It is vital to notify your doctor immediately if you start to develop
palpitations or dizzy spells, as this may be the first sign of cardiac involvement.
Your liver
Granulomas are present in the liver in 50 percent to 80 percent of patients
with sarcoidosis. However, patients usually do not notice the symptoms due to
liver involvement. Rarely, liver disease can progress to hypertension in the liver
(called portal hypertension) or cirrhosis (a disease causing widespread disruption of liver function).
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Your renal and endocrine system
Abnormalities can occur in the way the body handles calcium. For example,
hypercalcemia (an excess of calcium in the blood) occurs in 2 percent to 10
percent of patients. Hypercalciuria (an excess amount of calcium in the urine)
occurs in up to 21 percent of patients. Kidney stone formation, damage to the
structure of the kidney itself, and kidney failure also may occur.
Your reproductive system
Sarcoidosis can affect the male reproductive system, particularly the testes, and
may cause male infertility and erectile dysfunction. The disease rarely affects the
female reproductive system. Sarcoidosis does not increase the incidence of fetal or
maternal complications during pregnancy, unless it has caused severe impairment
of the heart or lungs; however, the disease may worsen after childbirth.
Your other organs
of note
Clinical Trials
Cleveland Clinic patients
have access to clinical
trials, should they qualify.
This provides them
treatments otherwise
Cytopenias (deficiencies in the amounts of certain blood cells) are the most
common “miscellaneous” symptom. Involvement of the spleen or bone marrow
may lead to anemia and other blood abnormalities. These conditions usually are
not of clinical significance. Bone thinning (osteopenia) also is common in sarcoidosis, as a result of the disease or as a side effect of prednisone therapy.
Some very common conditions seen in those with sarcoidosis are depression,
fatigue and obstructive sleep apnea. Often, these are the most significant problems affecting the quality of life. Depression may occur in up to two thirds of
patients with sarcoidosis, and can cause much of the fatigue that people with
sarcoidosis experience.
Sleep apnea is suspected in individuals with fatigue, excessive daytime sleepiness or an unrefreshed feeling on awakening in the morning. Most individuals
snore, or experience episodes of choking or gasping at night. Sarcoidosis of the
sinuses or nose, weight gain from steroids, and lupus pernio are three factors
that increase the chances for developing obstructive sleep apnea. If your doctor
suspects sleep apnea, it may be diagnosed by overnight monitoring in a sleep
lab (called a polysomnogram).
unavailable.
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How is sarcoidosis diagnosed?
Because the symptoms and laboratory findings associated with sarcoidosis can
occur in other diseases, there is no single test that can diagnose it. However, the
classic sign of the disease is the formation of granulomas (abnormal masses or
nodules consisting of inflamed tissue) in one or more of the major organs of the
body. Sarcoidosis-related granulomas are not different from granulomas that occur
in other diseases. As a result, a complete physical exam and medical history –
including occupational history, medication history, and environmental exposures
– must be made before concluding that the illness is, in fact, sarcoidosis.
The main tools your doctor will use to diagnose sarcoidosis include:
Chest X-rays – This test provides a picture of the lungs, heart and surrounding
lymph nodes, and reveals where infection-fighting white blood cells have formed
– often, a first indication of sarcoidosis. An X-ray also can show how much of the
lungs are affected by the disease.
Chest X-ray findings fall into one of the following five patterns described in
the chart on the next page. It is important to know that these X-ray patterns do
not represent disease stages (in other words, they are not the sequential steps
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in the disease course). The categories simply allow doctors to classify the
“types of disease.”
Bronchoscopy – Bronchoscopy involves passing a small tube (bronchoscope)
down the trachea (windpipe) and into the bronchial tubes (airways) of the lungs.
The purpose of this test is to inspect the bronchial tubes and to extract a biopsy
(a small tissue sample) to look for granulomas, and to rule out infection. Bronchoscopy is a safe, low-risk, outpatient procedure that provides your doctors
with a good chance of making an accurate diagnosis.
To prepare for this test, you will be asked not to eat or drink anything by mouth
for eight hours before the exam. Prior to the start of the exam, medications will
be given to you to help you relax. Because these medications can make you
groggy, an adult who can drive you home must accompany you. You will not be
allowed to drive. The procedure usually lasts 15 to 45 minutes, with several
additional hours for recovery.
Your doctor may perform a bronchoalveolar lavage (washing out the air sacs),
biopsies of the air sacs or airway wall using forceps, or biopsy of a lymph node
using a needle that is inserted through the bronchoscope. Guidance of the needle
with an ultrasound probe attached to the bronchoscope (endobronchial ultrasound
or EBUS) can almost always lead to a diagnosis of sarcoidosis when it is present.
CT scan – This test is another form of X-ray that provides an even more detailed
look at the lungs and lymph glands than that provided by a routine chest X-ray.
This test does not hurt and simply involves lying on a table for about 10 minutes.
Mediastinoscopy – This is a surgical procedure that involves a small incision
at the base of the neck through which an instrument is passed to biopsy lymph
nodes in the chest cavity. This test is performed under general anesthesia in the
operating room of a hospital and takes one to two hours (same day procedure).
The need for mediastinoscopy to make a diagnosis has gone down dramatically
as EBUS-guided biopsies have become widespread.
Pulmonary function (breathing) tests – These tests measure how well the lungs
are working (expanding and exchanging oxygen and carbon dioxide in the blood).
One pulmonary function test uses a device called a spirometer. This device records
the changes in air flow as a person inhales and exhales, as well as the overall volume of air exhaled. The development of granulomas and fibrosis of the lung tissue
stiffen the lung tissue and destroy the air sacs, making it more difficult for the
lungs to perform these tasks.
Other biopsies – In addition to the bronchoscopic biopsy or lymph node biopsy
by mediastinoscopy, tissue samples can be taken from any other involved site
including other lymph nodes, skin and other sites to determine where granulomas have formed.
Blood tests – Blood analyses evaluate the number and types of blood cells and
blood proteins in the body, and how well the cells are functioning. They also
track increases in calcium levels and abnormal liver function that sometimes
accompanies sarcoidosis. One blood test measures a substance called angiotensin-converting enzyme (ACE), which is secreted in large amounts by cells that
make up granulomas. ACE levels, however, are not always high in sarcoidosis
patients, and increased ACE levels also can show up in other illnesses. In short,
there is no specific blood test to diagnose sarcoidosis. A newer blood test that
is sometimes more useful than ACE is measurement of the soluble interleukin 2
receptor levels (sIL2R).
Cleveland clinic sarcoidosis center of excellence
Chest X-ray Patterns
Conditions they describe
0 = Normal chest X-ray
I = Bilateral hilar lymphadenopathy
(BHL) – the X-ray shows an equal
degree of enlargement of lymph
nodes at the “root” of both sides of
the lungs. This is a common presentation of sarcoidosis.
II = BHL plus pulmonary infiltrations – the X-ray shows a disease
process as described above with
expansion into and throughout additional lung tissue.
III = Pulmonary infiltration only
(without BHL) – the X-ray shows
a disease process that is spread
throughout the lung tissue (with no
enlargement of lymph nodes).
IV = Pulmonary fibrosis – the X-ray
shows small lung fields, scarring
and “retraction” of both hila (the
area at the “root” of the lungs). This
type of disease is the most severe or
permanent form of the disease.
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Pulse oximetry – This test measures the amount of oxygen in the blood by way of
a sensor attached to a patient’s finger. If the oxygen level is low, your doctor may
recommend the use of supplemental oxygen.
Electrocardiogram (EKG or ECG) – This is a routine office test that checks the
electrical activity of the heart. For this test, electrodes with adhesive pads are
attached to the skin of the patient’s chest, arms and legs. The EKG machine
creates a picture, on graph paper, of the electrical impulses traveling through
your heart. This screening test helps doctors detect several abnormalities in
the heart rhythm.
PET scan – In this test, a small amount of radioactive material called F-fluorodeoxyglucose is injected into a vein. This substance collects in the areas where
the granulomas have collected. A scanner then detects and records the location
and amount of inflammation in the body. This scan will detect inflammation created by conditions other than sarcoidosis, so it will be used in combination with
other testing.
Gallium scanning – In this procedure, the radioactive chemical gallium-67 is
injected into a vein. The gallium collects in inflamed body tissue. A scan of the
body then indicates which tissues and how much tissue is affected. The scan will
reveal any type of inflammation occurring in the body, however, and does not necessarily mean the patient has sarcoidosis. Because of this test’s limitations, it is
not commonly performed.
Purified protein derivative – This is a type of skin test that is used to help establish prior exposure or infection with tuberculosis (TB). Since TB is sometimes
confused with sarcoidosis, this simple test is frequently performed. In sarcoidosis,
this skin test is usually negative or nonreactive.
Slit-lamp examination – This examination looks at the inside of the eye and is
used to detect eye-related problems caused by sarcoidosis.
How is sarcoidosis treated?
There is no cure for sarcoidosis, but the disease may get better on its own over
time. Many people with sarcoidosis have mild symptoms and do not require any
treatment at all. Treatment, when it is needed, generally falls into two categories –
maintenance of good health practices and drug treatment.
Good health practices include:
• Getting regular check-ups with your healthcare provider
•S
taying up to date with your vaccinations, including influenza, pneumonia,
and for some patients, shingles vaccine.
• Eating a well-balanced diet with a variety of fresh fruits and vegetables
• Drinking eight to 10 eight-ounce glasses of water a day
• Getting six to eight hours of sleep each night
• Exercising regularly, and managing and maintaining your weight
• Quitting smoking
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•A
voiding exposure to dust, chemicals, fumes, gases, toxic inhalants and other
substances that can harm your lungs
• If you have had problems with high calcium levels, some patients should
avoid excessive amounts of calcium-rich foods (such as dairy products, oranges, canned salmon with bones), vitamin D and sunlight. Daily sunbathing is
an example of excessive sunlight and should be avoided; sunlight received
from activities of everyday living is acceptable. The proper amount of calcium
should be discussed with your doctor.
Drug treatments are used to relieve symptoms, reduce the inflammation of the
affected tissues, reduce the impact of granuloma development and prevent the
development of lung fibrosis and other irreversible organ damage.
of note
Corticosteroid treatment
controls the disease rather
than cures it. The symptoms
respond to treatment in the
majority of patients. A rela-
Corticosteroids are particularly effective in reducing inflammation, and are
typically the first drugs used in treating sarcoidosis. The oral corticosteroid
prednisone is the most commonly used corticosteroid.
tively high dose is usually
For patients with no symptoms or very mild symptoms, the side effects of
prednisone therapy may outweigh possible benefits, so treatment is usually not
recommended for this disease stage. Corticosteroids are more typically reserved
for patients with disease that is of moderate severity. Symptoms, especially
cough and shortness of breath, generally improve with steroid therapy.
by a slow taper to the lowest
prescribed at first, followed
effective dose.
Corticosteroid treatment controls the disease rather than cures it. The symptoms
respond to treatment in the majority of patients. A relatively high dose is usually
prescribed at first, followed by a slow taper to the lowest effective dose. Fortunately, disease relapses – when they occur – usually respond to retreatment with
steroids. Patients who improve and remain stable for more than one year after
stopping treatment have a low rate of relapse.
If steroids are prescribed, you should see your doctor at regular intervals so that
the disease and side effects of treatment can be monitored. The common side
effects of corticosteroids include:
• Excessive weight gain
• Insomnia
• Acne
• Diabetes in susceptible people
• High blood pressure
• Glaucoma
• Cataracts
• Osteoporosis
• Depression and emotional irritability
• Skin bruising
• Increased risk of infections
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Are there alternative treatments
to corticosteroids?
of note
It is important to
keep in mind that all
of the following
treatments have been
used and studied much
less extensively than
corticosteroids.
Other treatments are available for patients who cannot tolerate steroids either
because they are contraindicated or because side effects cannot be tolerated.
Patients whose disease does not respond to steroids or who wish to lower the
dose of steroids and use another drug in combination have additional treatment
options as well.
It is important to keep in mind that all of the following treatments have been
used and studied much less extensively than corticosteroids. Doctors with special
expertise in sarcoidosis should manage patients who are on regular prednisone
therapy or any of the following alternatives:
• Methotrexate, leflunomide azathioprine, mycophenolate: These medications,
most often used in rheumatoid arthritis, have been used in place of or in addition to corticosteroids to treat pulmonary sarcoidosis and chronic sarcoidosis.
Methotrexate, given as pills or as a skin injection, usually once per week,
has a small chance of causing irritation or damage to the liver. All of these
medications require several months to start working and will require regular
monitoring of blood tests.
•H
ydrochloroquine and chloroquine: These oral antimalarial drugs have been
used to treat sarcoidosis of the skin, lungs and nervous system. They are used
to treat the hypercalcemia seen with sarcoidosis. Patients on these drugs need
occasional monitoring of their eyes by an ophthalmologist.
•C
yclophosphamide: This medication is typically reserved for disease that
has reached the severe stage and after other therapies have failed. Cyclophosphamide is associated with many severe side effects, including bone
marrow suppression and kidney damage.
•P
entoxifylline and thalidomide: Some studies have reported beneficial effects
of these drugs in certain forms of sarcoidosis.
• Inflixamab or adalimumab: These medications, which are given as an intravenous injection once per month or under the skin (subcutaneous injection), have
been used for patients with severe sarcoidosis and for sarcoidosis causing neurologic symptoms. They usually are used after other options are not successful
or not tolerated.
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•C
olchicine: This oral medication, commonly used to treat gout, is sometimes
prescribed for the treatment of sarcoid arthritis.
•V
arious NSAIDs (nonsteroidal anti-inflammatory drugs, such as ibuprofen or
aspirin): These drugs may help reduce acute inflammation and relieve arthritis
and fever. They are not recommended for the treatment of sarcoidosis affecting
the lungs.
•T
opical corticosteroids: These agents could be used in several preparations
(for example, eye drops, skin creams and respiratory sprays) for mild local
symptoms of sarcoidosis. Although they are a lot safer than steroid pills, they
also are less effective.
•O
rgan transplantation: This last-resort option is considered in patients with
end-stage disease, where the kidneys, heart or lungs have failed.
When is treatment started?
Many questions exist regarding the appropriate timing and duration of treatment
for sarcoidosis. The decision to begin treatment generally depends on the organ
system involved and the severity of disease.
There are several situations, however, under which some form of treatment
is usually given right away. These include patients with neurologic, cardiac
and sight-threatening disease; those with serious pulmonary symptoms and/
or worsening lung function; and those with kidney involvement – specifically,
hypercalcemia. Because of the serious effects that can occur when these systems are involved, treatment is started even if symptoms are mild.
Other indications for which treatment could be considered include significant
impairment of quality of life as a result of fever, weakness, fatigue, joint pain,
nervous system changes, respiratory symptoms (especially shortness of breath
and cough) and disfiguring skin disease.
What can happen as the
disease progresses?
In many people with sarcoidosis, the disease appears briefly and then disappears
without the person even knowing they have the disease. When sarcoidosis seriously affects the ability of the lungs to function normally, patients may require
supplemental oxygen (supplied in a small portable oxygen tank and administered
through plastic tubing clipped to the nose) to help them breathe.
Twenty percent to 30 percent of people have some permanent lung damage.
For 10 percent to 30 percent, sarcoidosis is a chronic condition. In some people,
the disease may result in the deterioration of the affected organ.
When the granulomas or fibrosis seriously affect the function of a vital organ – such
as the lungs, heart, nervous system, liver, or kidneys – sarcoidosis can be fatal.
Death occurs in 1 to 5 percent of all patients with sarcoidosis and in 5 percent
to 10 percent of patients with chronic progressive disease. The leading cause of
sarcoidosis-related death in the United States is irreversible pulmonary fibrosis.
Cleveland clinic sarcoidosis center of excellence
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respiratory institute | sarcoidosis | treatment guide
Frequently Asked Questions
Q. If I think I might have sarcoidosis, what should I look for in selecting a doctor or treatment center?
A. Because the signs and symptoms of sarcoidosis can resemble other diseases, the diagnosis of sarcoidosis can be difficult
to make. Therefore, it would be wise to seek out a doctor and/or treatment center with a declared interest and expertise in
sarcoidosis diagnosis and treatment. Also, because the disease can affect multiple organs, the treatment center selected
should be capable of providing a comprehensive plan of care through a team of specialists. Finally, because the disease may
disappear and reappear over a lifetime, the healthcare provider selected should be one that is committed to a lifetime of
quality care and follow-up.
Q. Is there a cure for sarcoidosis?
A. There is no cure for sarcoidosis, but the disease may get better on its own over time or with drug therapy. Drug treatments
are used to relieve symptoms, reduce the inflammation of the affected tissues, reduce the impact of granuloma development,
and may prevent the development of lung fibrosis or other irreversible organ damage.
Q. Is sarcoidosis a type of cancer?
A. Sarcoidosis is not a form of cancer.
Q. Can my family get sarcoidosis from me?
A. Sarcoidosis is not contagious so your friends and family members won’t catch the disease from you.
Q. Is sarcoidosis a genetic disease? Will my children get it?
A. Within some individual families, the presence of sarcoidosis in a first- or second-degree relative has been shown to
increase the chance of getting the disease by nearly five-fold. Multiple genes have been identified that affect the chances
of getting sarcoidosis, and it is the combination of these genes that come together to influence susceptibility. Some of
the genes will be passed to offspring, which likely accounts for a large proportion of the elevated risk in close relatives.
Despite this, there is currently no screening test to identify who is at risk, and the likelihood of any given relative getting
the disease is still so low that screening for sarcoidosis is not currently recommended.
Q. Why does sarcoidosis affect blacks more often than other races or ethnic groups?
A. In addition to blacks – and black women in particular – sarcoidosis most commonly occurs in people of Scandinavian,
German, Irish and Puerto Rican descent. Genes are likely responsible for most of this phenomenon.
Q. Is sarcoidosis more common in certain areas in the United States?
A. Sarcoidosis appears to be more common in the South and Southeast, but why this is so remains unknown at the
present time.
Q. Will sarcoidosis affect my pregnancy?
A. Sarcoidosis itself should not interfere with your pregnancy or affect your unborn baby. Many women’s symptoms
improve while they are pregnant because the body produces a higher level of its own corticosteroids.
However, if the disease has caused significant organ damage, especially to the heart or lungs, it may be more difficult
to successfully carry the baby to term and could be dangerous for both the mother and the baby. This issue should be
discussed carefully with your doctor if you are contemplating pregnancy.
216.444.3613
Cleveland clinic sarcoidosis center of excellence
sarcoidosis | treatment Guide | respiratory institute
Q. Is my skin rash contagious?
A. No. The rash may not look pretty, but it cannot be passed to others through touch or other means.
Q. Can sarcoidosis develop into asthma or emphysema?
A. Although sarcoidosis commonly affects the lungs and can cause shortness of breath, wheezing and cough,
sarcoidosis does not cause emphysema nor does it develop into asthma.
Q. Do I have to get rid of my pets if I have sarcoidosis?
A. Not at all. You do not have to get rid of your family cat, dog, horse or other pet if you have sarcoidosis.
Q. Do I need to restrict my level of exercise?
A. There’s no need to restrict your activities simply because you have sarcoidosis. If you become out of breath,
stop and rest. But there is no reason to stop participation in athletic activities.
Q. Do I need to alter my diet in any way?
A. Not unless the sarcoidosis is causing high blood calcium levels. About one in 10 people with sarcoidosis do have high
blood calcium levels. If this is the case for you, reduce your intake of calcium-rich foods (such as dairy, oranges and canned
salmon with bones), vitamins containing calcium, vitamin D and avoid excessive sun exposure (such as sunbathing).
Q. Will drinking alcoholic beverages affect my disease?
A. Moderate social drinking has no effect on sarcoidosis.
Q. Will sarcoidosis affect my sex life?
A. Rarely, sarcoidosis can affect the endocrine glands or the reproductive organs in a way that affects sex life. More often,
sexual dysfunction is related to the presence of small fiber neuropathy or other factors like depression.
Q. What are the side effects of treatment with corticosteroids?
A. Common side effects of corticosteroids include excessive weight gain, insomnia, acne, diabetes in susceptible people,
high blood pressure, glaucoma, cataracts, osteoporosis, depression and emotional irritability, skin bruising and increased
risk of infections. Your doctor will discuss the side effects of corticosteroids and all drug treatments with you.
Q. I was recently diagnosed with sarcoidosis. What’s my outlook?
A. Most people with sarcoidosis live normal lives. About 60 percent of people with sarcoidosis recover on their
own without any treatment, 30 percent have persistent disease that may or may not require treatment, and up to
10 percent with progressive long-standing disease have serious damage to organs or tissues that can be fatal. The
rate of death from sarcoidosis historically has ranged from 1 percent to 5 percent in various studies, but is likely
closer to the low end of that range.
Q. Where can I get more information?
A. Your doctor is the best resource for finding out important information related to your particular case. Not all patients
with sarcoidosis are alike, and it is important that someone who knows you as a whole person evaluates your situation.
Because sarcoidosis can affect different organ systems and can change over time, it is important for patients with
sarcoidosis to see their doctors at regularly scheduled intervals.
Cleveland clinic sarcoidosis center of excellence
216.444.3613
respiratory institute | sarcoidosis | treatment guide
Why choose Cleveland Clinic?
At Cleveland Clinic’s Sarcoidosis Center of Excellence, our team of pulmonologists,
cardiologists, electrophysiologists, neurologists, ophthalmologists, dermatologists
and rheumatologists offer comprehensive and personalized care, using the latest
technology.
Our team also is constantly striving to understand the disease process of sarcoidosis and to find alternative treatments for its care through our research. We offer
patients access to clinical trials not widely available at other institutions.
Making an appointment
Call 216.444.6503 to make an appointment at Cleveland Clinic’s Sarcoidosis
Center of Excellence.
Need a second opinion,
but cannot travel to Cleveland?
Our MyConsult service offers secure online second opinions for patients who
cannot travel to Cleveland. Through this service, patients enter detailed health
information and mail pertinent test results to us. Then, Cleveland Clinic experts
render an opinion that includes treatment options or alternative and recommendations regarding future therapeutic considerations. To learn more about MyConsult,
please visit clevelandclinic.org/myconsult.
216.444.3613
Cleveland clinic sarcoidosis center of excellence
sarcoidosis | treatment Guide | respiratory institute
Notes
Cleveland clinic sarcoidosis center of excellence
216.444.3613
12-PUL-131
216.444.6503
Cleveland clinic sarcoidosis center of excellence