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 vegetables.
- 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 organ damage.
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 steroid therapy.
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's 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 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 can be taken as pills or an injection under the skin, once per week. All of these medications can suppress the immune system and have the potential to cause liver toxicity so blood work needs to be monitored every 4-8 weeks. In addition, these drugs have the potential to harm a fetus (unborn child) so pregnancy needs to be avoided. Patients who take methotrexate must not drink alcohol.
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 every 4-8 weeks or under the skin (subcutaneous injection) every 1-2 weeks, have been used recently for patients with severe sarcoidosis. They are usually used after other options are not successful or not tolerated. They significantly increase the risk for infections.
Various NSAIDs (nonsteroidal anti-inflammatory drugs [such as ibuprofen or aspirin]): These drugs may help reduce acute inflammation and relieve arthritis and fever.
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.