SARCOIDOSIS

Sarcoidosis is a disease which occurs when areas of inflammation grow in different organs of the body. Very small growths called granulomas are associated with it and occur in the lungs, lymph nodes, eyes, skin, and spleen. These growths may clear up on their own or cause permanent damage.

The cause of sarcoidosis is unknown. For some reason the body's immune system acts as if it needs to fight an invader.

Sarcoidosis occurs more frequently in Caucasians of European descent and among African-Americans. About 10-40 out of every 100,000 people develop sarcoidosis. It is most common in young people between the ages of 20 and 40. Sarcoidosis is not contagious.

What are the signs and symptoms?

People with sarcoidosis may have no symptoms, only vague symptoms of a general nature such as weight loss and fever, or symptoms associated with a specific organ, usually the lungs. More than

one organ can be involved. Up to half of sarcoidosis patients have no symptoms when the illness is diagnosed. Thus, the diagnosis can be difficult to make. Signs and symptoms associated with specific organ involvement can include the following:

Lungs
Inflammation in the lungs can cause shortness of breath and a cough (usually a dry cough). In some people, the symptoms go away; in others there can be permanent scarring of the tissue.

Lymph Nodes
Enlargement of various lymph nodes can occur.

Eyes
Inflammation of the eye is common and can lead to watering, redness, and sensitivity to the light. Blurred vision can also occur. In many cases there can be eye involvement with no obvious visual problems; therefore, it is important that the person be checked regularly by an eye doctor.

Skin
Skin involvement may appear as raised, pink or purplish areas or as painful nodules under the skin.

These deeper nodules are generally found on the legs and are often associated with arthritis (from sarcoidosis as well).

Bone
Nodules in the bone can be painful and can cause problems in the hands and feet.

Spleen and Liver
Enlargement of these organs can occur.

Heart
This occurs only in a small percentage of people and can be difficult to diagnose. Heart involvement can include heart rhythm abnormalities and can affect the ability of the heart muscle to pump blood.

Brain and Nervous System
Only about one in 100 people with sarcoidosis are affected. Nodules can infiltrate the brain and the nerves and cause many symptoms, including loss of sensation, loss of muscle strength, headaches, and dizziness.

How is the diagnosis made?

The inflamed microscopic growths (granulomas) seen in the lungs with sarcoidosis are similar to those in other diseases such as tuberculosis, fungal diseases, berylliosis, and farmer's lung. Because of this, a careful evaluation is necessary to make a diagnosis. Only after the known causes of granulomas have been "ruled out" is the diagnosis of sarcoidosis made. An evaluation to detect sarcoidosis should include the following:

Thorough Medical Examination
This is done to "rule out" other diseases that may present as sarcoidosis.

Chest X-Ray
Doctors look at the x-ray for evidence of enlarged lymph nodes and small round spots in the lung caused by the inflamed growths. "Staging" or identifying the degree of lung involvement can help the doctor determine the progress of the sarcoidosis. A scale of 0-III is commonly used with III having the highest amount of lung involvement. In a few cases, only the x-ray is needed to make the diagnosis.

Pulmonary Function Tests
Standard breathing tests give an indication of the severity of lung disease. There is nothing unique about sarcoidosis on these tests, so they do not substitute for other, more specific tests. These tests can show obstruction of air flow out of the lungs, restriction of the lung's ability to take in air, and a decrease in the transport of oxygen from the lung into the blood stream. The most important types of breathing tests in sarcoidosis are spirometry, lung volumes, and diffusing capacity. In some cases, measurement of blood oxygen levels during an exercise test can also be done.

Tissue biopsy
A microscopic examination of tissue samples from the inflamed growths in the lungs is needed to be absolutely sure of the diagnosis. Bronchoscopy, an out-patient procedure in which the doctor introduces a narrow tube through the nose and into the airways, can be performed to obtain this tissue. Sometimes the diagnosis is made by obtaining tissue samples from the skin, liver, or enlarged lymph nodes.

Bronchoalveolar Lavage
At the time that bronchoscopy is done, a small part of lung can be washed (lavaged), in order to obtain some cells of the immune system from the lung. By counting the types of cells in lavage fluid, it is possible to get an estimate of how inflamed the lungs are, and whether the type of inflammation is characteristic of sarcoidosis. This procedure is in routine use at a number of medical centers (including National Jewish Medical and Research Center), and also provides a great deal of information for research into this disorder.

Eye Examination
A slit lamp examination is an important part of an eye examination to detect inflammation.

Serum Angiotensin Converting Enzyme Level (S.A.C.E.)
If the level of this blood test is high, it can indicate the presence of sarcoidosis. It can sometimes be used to monitor if the sarcoidosis is improving or worsening. Unfortunately, other diseases can cause increased S.A.C.E. blood levels also, so this test cannot be used alone to make the diagnosis.

Computerized Tomography (CT Scan)
The CAT Scan makes it possible to see lymph nodes and scars in the lung when regular chest x-rays sometimes cannot.

Gallium Scan
The Gallium scan is a type of x-ray. A small amount of radioactive material is injected into a person's blood which travels to areas of the body that are inflamed. An x-ray camera scans the

body to see where the radioactive material has collected. Although not needed in all cases, gallium scanning can help show sarcoidosis in many organs, including the lungs, salivary glands, bone, spleen, and liver. This can lessen the need for tissue biopsies of all these organs.

Monitoring of Calcium Levels in the Blood and Urine
People with sarcoidosis can have sensitivity to vitamin D which results in too much calcium being absorbed through the intestines. This can cause a high level of calcium in the blood and urine. Exposure to the sun can further stimulate this process.

Kveim Test
The Kveim test is a test in which an extract of sarcoid affected tissue is injected under the skin. For several reasons, it is not considered part of a routine evaluation for sarcoidosis.

What is the treatment?

Not everyone with sarcoidosis will need treatment. One half to three quarters of the people diagnosed with sarcoidosis improve without treatment. Those who do not are usually placed on medication to reduce inflammation. Many people will recover, but a some will get worse despite treatment. The goal of treatment is to maintain good lung function, lessen symptoms, and prevent organ damage.

Corticosteroids, which work against inflammation, are the main treatment. Generally, prednisone (a tablet) is given daily or every other day, depending on the symptoms. It can decrease symptoms, improve lung function, decrease S.A.C.E. levels, reduce granuloma formation, and possibly, lessen scarring of the lungs. Prednisone can be associated with a number of side effects. Because of this, people on steroids should be carefully monitored by a doctor. The potential benefits from treatment usually outweigh the risks from the medication side effects.

What Other Treatment is Used?

The use of inhaled steroids (such as Azmacort®, Aerobid®, Vanceril®, Beclovent®) is also being tried. Although still considered experimental, this form of treatment may be effective in some people, and it has a lower risk of steroid side effects.

When steroids can't be used, other medications can be tried. These other medications are not used often, since their effect on sarcoidosis is not well understood and not predictable. They too can have side effects. These medications include: indomethacin, azathioprine, chloroquine, methotrexate, clyclosporine, and levamisole.

For a small percentage of people who develop chronic, progressive sarcoidosis, pulmonary rehabilitation and oxygen therapy may be needed.

One important thing a person can do to improve the outcome of this disease is to see a doctor when the symptoms first appear. This can help prevent damage to the lungs, eyes, heart, and other organs. People with sarcoidosis can, like others, develop lung disease from smoking. Therefore, it is also important not to smoke.