Sarcoidosis

Sarcoidosis is an inflammatory disease with no known cause and no known cure.

Description

Sarcoidosis is a disease in which inflammatory cells (granulomas) form usually in the lungs but it can affect the heart, liver, lymph nodes, skin, and eyes. Granulomas may collect and grow larger or form groups of granulomas. Granulomas usually affect more than one organ.

During the active phase of the disease, granulomas form and grow and symptoms may develop. Scar tissue may form in the affected organ(s). Sometimes the granulomas change an organ's structure and function.

During the nonactive phase, the inflammatory response decreases and the granulomas may shrink. Scars may remain and continue to cause symptoms.

The disease may resolve spontaneously within two to three years or there may be only an occasional flare-up of symptoms. In some people, the disease may get worse and cause permanent damage.

Demographics

Women ages 20–40 are more likely to develop sarcoidosis. Sarcoidosis occurs more often in people of African or Scandinavian descent (in particular Swedes and Danes). It occurs more often in nonsmokers than in smokers. In the United States certain groups have a higher percentage of cases of sarcoidosis including health care workers, naval aircraft servicemen, and firefighters.

African Americans have a four to seventeen time chance of developing sarcoidosis compared to Caucasians. Having a brother or sister or parent with sarcoidosis increases the risk of developing the disease five fold.

Causes and symptoms

The cause of sarcoidosis is unknown. It is theorized that sarcoidosis develops when the body's immune system responds abnormally to bacteria, viruses , dust, or chemicals in the environment. Sarcoidosis may also develop as a result of overactivity of the body's immune system (autoimmune response). There may also be a genetic predisposition to developing sarcoidosis.

Many people have no symptoms. Symptoms depend on which organs are affected. The most common symptoms include shortness of breath, persistent dry cough, wheezing, and reddish bumps or rashes on the skin. Eye symptoms may include vision problems and dry eyes. Other symptoms rarely occur but may include a generalized sick feeling (malaise), loss of appetite, weight loss , and fatigue. Blacks tend to have the more severe and chronic form of the disease.

Diagnosis

Evaluation to diagnose sarcoidosis begins with a complete medical history and physical examination. Diagnostic testing may include a chest x-ray, pulmonary function testing, tissue biopsy, an eye examination , the Serum Angiotensin Converting Enzyme (S.A.C.E.) blood test, computed axial tomography (C.A.T. scan), Gallium scanning, and calcium blood and urine level testing.

Chest x-ray is used to detect the presence of enlarged lymph nodes and granulomas. Pulmonary testing using a special instrument called a spirometer evaluates lung volumes and assesses the lungs' ability to exchange oxygen and carbon dioxide. Tissue biopsy is used to obtain tissue samples to confirm a diagnosis of sarcoidosis. Tissue samples are obtained during a bronchoscopy to examine for the presence of granulomas. Bronchoscopy is an outpatient procedure during which a specially trained physician inserts a narrow tube through the nose into the lungs. Bronchoalveolar lavage may also be done during bronchoscopy. During bronchoalveolar lavage, part of the lung is washed (lavaged) then the fluid is examined for cell inflammation characteristic of sarcoidosis. If the physician suspects that other parts of the body are affected by sarcoidosis, e.g. the skin, lymph nodes, or eyes, a tissue sample for biopsy may be taken from these areas.

Eye examination is conducted by an ophthalmologist to evaluate for inflammation or damage to the eyes from sarcoidosis. If the S.A.C.E. blood test result is elevated, it may indicate the presence of sarcoidosis. This blood test may be used at periodic intervals to determine if the disease is getting worse or improving. For some people with sarcoidosis, calcium levels are elevated in the blood and urine therefore, calcium blood and urine testing may be done.

C.A.T. scanning may reveal enlarged lymph nodes and lung scarring that a chest x-ray cannot. During Gallium scanning, a small amount of radioactive material is injected into the bloodstream. The radioactive material collects in inflamed areas of the body which can help to show sarcoidosis in the lungs and other organs.

Treatment

Treatment may be unnecessary if symptoms are mild. If treatment is needed it usually consists of corticosteroids to decrease inflammation. Prednisone is the corticosteroid used most often. The length of time medication is needed is dependent on how severe the disease is and how quickly the symptoms improve. Corticosteroids can reduce symptoms, improve lung function, decrease S.A.C.E. levels, decrease granuloma formation, and may reduce lung scarring.

Corticosteroids can cause serious side effects especially when taken for an extended period of time. These side effects include osteoporosis (a disease in which bones become thin and brittle), high blood pressure , cataracts , diabetes, increased risk of infection, and weight gain with redistribution of body fat. Benefits of using corticosteroids outweigh medication side effects.

Lung function testing and chest x-ray should be done every six to twelve months. An annual eye examination and electrocardiogram (E.C.G.) are recommended.

Nutrition/Dietetic concerns

Maintain a healthy weight. It is important to eat a balanced diet and drink adequate amounts of water daily. Foods high in calcium should be avoided. Foods high in calcium include dairy products, oranges, and canned salmon with bones.

Therapy

Don't smoke. Remain physically active without overexertion. Get adequate rest. Avoid exposure to chemicals, dust, toxic fumes, and other environmental lung irritants. Get recommended follow-up testing and regular medical care. Self-help groups are available for people diagnosed with sarcoidosis. Medication should be taken as prescribed.

Prognosis

Many people diagnosed with sarcoidosis do not require treatment and improve spontaneously. Those with advanced lung disease or heart or neurological involvement have a poorer prognosis.

Prevention

Because there is no definitive cause for sarcoidosis, there is no way to prevent it.

Caregiver concerns

Regular follow-up care is vital as new symptoms may occur unexpectedly and the disease may worsen without notice. It is important to remember that sarcoidosis is not contagious.

QUESTIONS TO ASK YOUR DOCTOR

• Will I need treatment?
• What are some of the treatments that may be recommended?
• How will I know if the treatments are working?
• Are there any side effects to the treatment?
• Do I need to see a specialist?

Ensure that medication is taken as prescribed. Some medications used to treat sarcoidosis can have side effects so it is important to be aware of potential adverse effects. Report them to your health care provider as indicated.

June G. Borazjani R.N., M.S.N., C.P.H.Q.