How Is Sarcoidosis Treated?

The goals of treatment are to:

• Improve how the organs affected by sarcoidosis work
• Relieve symptoms
• Shrink the granulomas.

Treatment may shrink the granulomas and even cause them to disappear, but this may take many months. If scars have formed, treatment may not help, and you may have ongoing symptoms.

Your treatment depends on:

• What symptoms you have
• How severe your symptoms are
• Whether any of your vital organs (e.g., your lungs, eyes, heart, or brain) are affected
• How the organ is affected.

Some organs must be treated, regardless of your symptoms. Others may not need to be treated. Usually, if you don't have symptoms, you don't need treatment, and you probably will recover in time.

Drugs

The main treatment for sarcoidosis is prednisone. Prednisone is a corticosteroid, or anti-inflammatory drug. Sometimes it is used with other drugs. Sometimes other corticosteroids are used.

Prednisone almost always relieves symptoms of inflammation. If a symptom doesn't improve with prednisone treatment within a couple of months, consult your physician.

Prednisone is usually given for many months, sometimes for a year or more.

Low doses of prednisone can often relieve symptoms without causing major side effects.

When used at high doses, prednisone can cause serious side effects.

Side effects can include:

• Weight gain.
• Diabetes.
• High blood pressure.
• Mood swings (depression).
• Difficulty sleeping at night.
• Heartburn.
• Acne.
• Thinning of the skin and bones (called osteoporosis).
• Cataracts.
• Glaucoma.
• Adrenal gland insufficiency, which occurs when these glands don't make enough of certain hormones. This requires treatment by an endocrinologist (en"do-kri-nol'o-jist), a doctor who specializes in the diagnosis and treatment of the endocrine glands. The endocrine glands include your adrenal and pituitary glands.
• Aseptic (a-sep'tik) or avascular (ah-vas'ku-lar) necrosis (ne-kro'sis) of the hip, the development of cysts and hardened and dead tissue in the hip.

Your doctor can usually help you manage these side effects.

When it is time to stop taking prednisone, you should cut back slowly, with your doctor's help. This will help prevent flare-ups of sarcoidosis and allow your body to adjust to life without the drug.

You may also want to see an endocrinologist to make sure that your endocrine glands are making enough hormones. The endocrinologist may prescribe certain hormones for you to take until your endocrine glands are working well again.

Other Drugs Used To Treat Sarcoidosis

Other drugs are sometimes used to treat sarcoidosis. Your doctor may prescribe one of them if:

• Your condition gets worse while you are taking prednisone
• You can't stand the side effects of prednisone.

Most of these other drugs are immune system suppressants. This means that they prevent your immune system from fighting things like bacteria and viruses. As a result, you may have a greater chance of getting infections.

Most of these drugs also can cause serious side effects. Some also could increase your chances of getting cancer, especially if you take them at high doses.

You and your doctor must weigh living with the symptoms of sarcoidosis against the side effects of the drugs.

Some drugs work better than others for different people.

You may be given more than one drug.

Some drugs used to treat sarcoidosis are taken by mouth. Others are applied locally to an affected area.

Local therapy is the safest way to treat sarcoidosis. The drug is applied directly to the affected area. As a result, only small amounts of the drug reach other parts of your body.

Drugs used for local therapy include:

• Eye drops
• Inhaled drugs for your lungs
• Skin creams.

Drugs can be used locally only if the affected area is easily reached. For instance, inhaled steroids can ease coughing and wheezing in the upper airways, but they don't seem to relieve these symptoms when the affected lung tissue is deep within your chest.

Talk with your doctor about these treatments and the side effects that may occur.

The other drugs used to treat sarcoidosis include:

• Hydroxychloroquine (Plaquenil). This drug can usually help people who have sarcoidosis in the skin or a high level of calcium in their blood. This drug can irritate your stomach.
• It also can cause eye problems. Before starting on this drug, you should see an ophthalmologist (of"thal-mol'o-jist), or eye doctor, for some baseline tests. Once you start taking it, you should have your eyes examined every 6 months.
• Methotrexate. This drug is taken once a week by mouth or injection and usually takes up to 6 months to relieve symptoms.
• This drug may cause side effects, especially if you take high doses. These include:
  • Nausea.
  • Mouth sores.
  • A decrease in infection-fighting white blood cells. You then have a greater chance of getting an infection. If you take this drug, you should have regular blood tests to check the levels of your white blood cells.
  • An allergic reaction in your lungs that goes away when you stop taking the drug. This is extremely rare.
  • Liver damage. This is the most serious side effect. If you take methotrexate you should be followed regularly by your physician.
  If you are pregnant, you should not take this drug.
• Taking folic acid can help you reduce your chances of having bad side effects from methotrexate.

• Azathioprine (Imuran). This drug may work in about half of the people who have sarcoidosis. You usually take it for at least 6 months. Side effects include:
  • Nausea
  • Reduced white blood cell levels, which increases your chances of getting an infection.
  • This drug has caused cancer in some people, especially when they have taken it at high doses.
  • If you are pregnant, you should not take this drug.
• Cyclophosphamide (Cytoxan). This is a very toxic drug. It is rarely used to treat sarcoidosis. It is given only to people who have serious forms of sarcoidosis, such as sarcoidosis in their central nervous system (neurosarcoidosis).
• This drug is more likely to cause nausea and reduce your white blood cell levels than either methotrexate or azathioprine. Your doctor should check your white blood cell levels often while you are taking this drug to make sure you have a high enough level to fight infection.
• Cyclophosphamide can also irritate your bladder. Some people who have taken it for more than 2 years have developed bladder cancer.
• If you are pregnant, you should not take this drug.
• Cyclophosphamide can be given intravenously (through one of your veins), which lessens some of its side effects, but this doesn't reduce the risk of cancer.

Treatments for Specific Types of Sarcoidosis

• Eyes. Sarcoidosis in your eyes almost always responds well to treatment. Often, the only treatment you need is eye drops containing corticosteroids. You should have yearly eye exams, even if you think your eyes are doing well.
• Spleen. Sarcoidosis can cause your spleen to become larger. This can lead to a decrease in your red or white blood cells or platelets and increase your chances of infection and blood clotting disorders. Treatment is usually given to increase the number of your blood cells and ease your pain. In rare cases, your spleen may need to be removed.
• Liver. Sarcoidosis rarely causes permanent liver damage. As a result, your liver usually isn't treated unless it's causing major symptoms (e.g., fever). Drug treatment can usually reduce granulomas in your liver. Liver transplantation has been successful in those rare cases in which the condition has become worse.
• Followup care includes regular blood tests to find out how well your liver is working. You should check with your doctor to find out how often you need these tests.
• Nervous system. Sarcoidosis in your nervous system (neurosarcoidosis) usually needs treatment. Nerve tissue heals slowly, so treatment often takes a long time. You may need to take several drugs at high doses.
• Erythema nodosum. These painful bumps on your shins often go away in weeks to months without treatment. Your doctor probably will not give you medication unless you are very uncomfortable. Aspirin or ibuprofen, an anti-inflammatory drug that you can buy without a prescription, will usually help.
• Heart. Sarcoidosis in your heart is usually treated with steroids. You may also be given heart drugs to improve your heart's pumping ability or to correct a disturbed heart rhythm.
• If you have a severe heart rhythm disturbance, your doctor may prescribe one of these devices:
  • A cardiac pacemaker, a small battery-operated device, often put under your skin, that regulates your heartbeat
  • A defibrillator, an implanted device that shocks your heart into a normal heartbeat or, if it has stopped, into beating.
  If your heart is severely affected and doesn't respond to treatment, a transplant may be done. But this is rarely needed.
• Lupus Pernio. This rash on your face, especially your cheeks and nose, can be distressing because it's in a very visible area. It often occurs with loss of your sense of smell, nasal stuffiness, and sinus infections.
• Options for treatment include:
  • Local treatment with skin creams
  • Oral drugs (plaquenil or prednisone, for example)
  • Local injections of steroid preparations.
  Lupus pernio is often treated by dermatologists, doctors who specialize in skin diseases, working with a sarcoidosis specialist.

Because sarcoidosis varies so much among different people, your doctor may find it hard to tell whether the treatment is helping.

Other Drugs Being Studied for Possible Use in Treating Sarcoidosis

Scientists also are studying drugs that are used for other conditions to see if they can help people who have sarcoidosis. These drugs include:

• Etanercept (Enbrel). This drug is an immune system suppressant. It's injected under the skin to reduce symptoms of rheumatoid arthritis. It may also be used to treat psoriasis (so-ri'ah-sis) or ankylosing spondylitis (ang"ki-lo'sing spon"di-li'tis), a type of arthritis that affects the joints in the spine. Early studies suggest that it will not be useful in treating sarcoidosis, but research is ongoing.
• Infliximab (Remicaide). This drug is an immune system suppressant. It's injected into a vein in your arm. It's used to treat Crohn's Disease, rheumatoid arthritis, and ankylosing spondylitis. Some studies have shown it to help sarcoidosis patients who also have lupus pernio, eye disease, or neurosarcoidosis. This drug has serious side effects but may improve lung function in some people who aren't helped by corticosteroids. More research is needed.
• Pentoxifylline. This drug is an immune system suppressant. Stomach and gastrointestinal side effects are common. Early studies show that it has helped some people who have sarcoidosis in their lungs reduce their doses of prednisone while taking it. More research is needed.
• Tetracycline. Tetracycline antibiotics are used to treat Lyme Disease, some types of pneumonia, and acne. A few small studies suggest that they may help in treating sarcoidosis in the skin. Research is ongoing.
• Thalidomide. This immune system suppressant can cause bad side effects. It is effective against other conditions that involve granulomas of the skin (e.g., leprosy, tuberculosis). Scientists are studying this drug to see if it can be used to treat sarcoidosis in the skin. More studies are needed.

What Does the Future Hold?

Scientists worldwide are trying to learn more about sarcoidosis and how to improve its diagnosis and treatment. Some recent studies have led to possible new treatments, which, in turn, are being studied. Current research includes studies of:

• The agent or agents that cause sarcoidosis
• Why sarcoidosis seems to act differently in people of different races
• Why sarcoidosis appears in some families
• How genes, passed from one generation to another, may make some people more likely than others to develop sarcoidosis
• How cells act and communicate with each other to cause sarcoidosis symptoms.