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 health care provider
- Eating a well-balanced diet with a variety of fresh fruits and
- Drinking 8 to 10 8-ounce glasses of water a day
- Getting 6 to 8 hours of sleep each night
- Exercising regularly, and managing and maintaining your weight
- Quitting smoking
- Avoiding exposure to dust, chemicals, fumes, gases, toxic inhalants, and
other substances that can harm your lungs
- Avoiding 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
advice in this bullet point is limited to patients with high blood or urine
levels of calcium.)
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
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.
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
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.
Results of some long-term studies indicate patients
can expect about a 10% improvement in symptoms up to five years after quitting
corticosteroid treatment. Whether or not this is a large enough benefit to
outweigh the risks of corticosteroid treatment remains an issue debated by
doctors. If steroids are prescribed, the patient should see his or her 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
- Diabetes in susceptible people
- High blood pressure
- Depression and emotional irritability
- Skin bruising
- Increased risk of infections
Are there alternative treatments to 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 or azathioprine: 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 usually once per week, has a small chance of
causing irritation or damage to the liver.
- Hydroxychloroquine 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.
- Cyclophosphamide or chlorambucil: These medications are usually used
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.
- Pentoxifylline and thalidomide: Recent studies have reported
beneficial effects of these drugs in treatment-resistant lupus pernio.
- Infliximab or adalimumab: These medications, which are given as an
intravenous injection once per month or under the skin (subcutaneous
injection), have been used recently for patients with severe sarcoidosis.
They are usually used after other options are not successful or not tolerated.
- Colchicine: This oral medication, commonly used to treat gout, is
sometimes prescribed for the treatment of sarcoid arthritis.
- Various 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.
- Topical 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 are also less effective.
- Organ transplantation: This option is rarely considered in patients
with end-stage disease, where the kidneys 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. These include patients with
neurological, heart, 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 an inability to work 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% of people have some permanent
lung damage. For 10% to 30%, sarcoidosis is a chronic condition, with symptom
progression despite treatment that has continued for more than two years. 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 6% of all patients with
sarcoidosis and in 5% to 10% of patients with chronic progressive disease. The
leading cause of sarcoidosis-related death in the United States is irreversible pulmonary fibrosis.
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