Sarcoidosis (SAR-coy-DOH-sis) is an inflammatory disease that can produce many different symptoms. It may affect the lungs, skin, joints and eyes, but can also involve almost any other organ or tissue in the body. The disease is characterized by granulomas. These are small rounded outgrowths made up of blood vessels, cells and connective tissue. Granulomas ordinarily are formed in response to chronic infection or inflammation or to a foreign substance.

Because sarcoidosis can affect almost any organ system, it mimics other rheumatic diseases capable of causing fever, arthritis, uveitis (eye inflammation), myositis (muscle disease) and skin eruption (rash). Sarcoidosis affects people differently. Some people may experience several symptoms; many people experience only a few. Some people may have an acute form of sarcoidosis -- severe, but short-lived. Others may have a chronic form, which can last several years or even a lifetime.

Sarcoidosis is most common in young adults between the ages of 20 and 40. It occurs in both men and women, although women are affected slightly more often. In the United States, it affects African-Americans more often and more severely than Caucasians.



WHAT CAUSES SARCOIDOSIS?

The cause of sarcoidosis is unknown. Suspected causes include:

a viral or bacterial infection;
a defect in the body's immune system;
an unidentified toxic substance
an unknown environmental cause;
an inherited or genetic factor.




WHAT HAPPENS IN SARCOIDOSIS?

With some people, sarcoidosis begins with no symptoms at all. A chest X-ray may be necessary to detect the condition. With other people, sarcoidosis begins with a few symptoms, then gradually becomes more widespread and serious. With still others, it begins with many symptoms that appear either gradually or very suddenly.



SKIN PROBLEMS

Granulomas form on the skin of about 10 to 35 percent of people with sarcoidosis. Lupus pernio is one kind of granuloma, that appears on the face, especially on the nose, cheeks and ears. This type of granuloma can damage skin tissue and leave scars.

Another type of skin granuloma occurs on the face, hands, feet, buttocks and shoulders. However, it usually clears up without leaving scars. Granulomas also can form in the lungs, muscles, lymph nodes, liver, spleen, joints, bone marrow and nervous system.

Erythema nodosum is another skin condition that can occur with sarcoidosis. It is a sudden, painful skin reaction characterized by tender red bumps, about the size of a quarter or larger, that suddenly appear on the shins. Erythema nodosum can be accompanied by arthritis of the ankle (and sometimes other joints) and a low-grade fever. It usually lasts from one to three weeks, and may recur. It occurs in about 10 percent of people with sarcoidosis and in about 50 percent with the acute form, particularly those with swollen lymph nodes in the chest.



LUNG PROBLEMS

Sarcoidosis often affects the lungs. Symptoms can include a dry cough, shortness of breath and/or chest pain. Lymph gland enlargement around the trachea and large bronchi can be seen on chest X-rays. Lung problems related to sarcoidosis generally go away on their own.

Fortunately, most people experience little or no breathing problems, and few people develop severe lung disease. However, symptoms should not be ignored. See your doctor if you have any of these symptoms.



EYE PROBLEMS

Sarcoidosis frequently affects the eyes. Inflammation or swelling of the iris (colored portion of the eye) can occur. This is called iridocyclitis. Symptoms include redness of the eye, pain and sensitivity to light. If not treated properly, blindness can result. Another condition, uveitis, can occur when there is inflammation in any part of the uveal tract, which is the pigmented layer of the eye. Tear glands also can become inflamed, causing sicca syndrome (dry eyes). Glaucoma (pressure inside the eye that can result in vision loss) also may occur. If you notice any change in your eyes, notify your doctor immediately.



ARTHRITIS

Arthritis means joint inflammation (arth = joint; itis = inflammation). It causes pain, warmth, redness and stiffness in the joints. Arthritis occurs in about one-sixth of people with sarcoidosis. The most common form of sarcoid arthritis is the acute form. It is characterized by:
erythema nodosum;
enlarged lymph glands;
joint involvement, mainly of the ankles andknees;
maximum intensity that occurs within a short period of when the disease begins;
limited movement;
moderate to marked inflammation;
absence of joint deformity or destruction;
duration from two weeks to four months.
Chronic sarcoid arthritis is much less common and is characterized by:
involvement of just one or a few joints (occasionally many joints), usually the knees, ankles and/or hands; possible joint destruction and disability;
dactylitis, a condition in which the fingers or toes become inflamed and take on a sausage-like appearance (granulomas in finger bones can contribute to this appearance.



OTHER GENERAL SYMPTOMS

Other general symptoms that people with sarcoidosis may experience include fever, weight loss and fatigue. A common observation people with sarcoidosis make is that they simply do not feel like themselves.



HOW IS SARCOIDOSIS DIAGNOSED?

Doctors have several ways of detecting sarcoidosis. During a physical exam, the doctor may discover enlarged, tender lymph glands, a dry cough and/or erythema nodosum. If eye problems are suspected, an ophthalmologist (eye specialist) may need to conduct a complete eye examination.

In most cases, chest X-rays will detect nodules or other abnormalities in the lungs. These may be found even in people who have no obvious symptoms. X-rays can detect other abnormal signs in the lung and chest resulting from sarcoidosis, including enlarged lymph nodes.

Another useful examination -- a biopsy -- involves the removal (under anesthesia) of a tiny piece of tissue from the skin lesions, lymph nodes or lungs. After the piece of tissue is removed, it is examined under a microscope to see if granulomas are present. Blood tests are another way of determining whether body organs are functioning properly, as well as ruling out other diseases. The ACE (angiotensin converting enzyme) test is a special blood test used to help diagnose sarcoidosis.



HOW IS SARCOIDOSIS TREATED?

Treatment depends on which symptoms are present and how severe they are. People with the acute form of sarcoidosis may need treatment to relieve discomfort from such symptoms as skin nodules, joint inflammation and muscle pain. The treatment for joint inflammation includes medication to reduce inflammation and physical therapy to maintain function. Medications used to treat inflammation include salicylates and nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs not only treat joint inflammation, but also help relieve pain. For people who have more troublesome symptoms or whose disease has spread to internal organs, corticosteroids also may be needed. These drugs reduce pain and inflammation and help keep granulomas from forming. Doctors may prescribe corticosteroids for patients who do not respond to aspirin or other NSAIDs.

Still other medications have shown promise in the treatment of sarcoid arthritis. Oral colchicine (COAL-chi-sin) is another type of medication prescribed for acute sarcoid arthritis. Studies indicate that methotrexate given by mouth in low doses once a week may be useful for lung problems. Chronic arthritis and skin involvement may benefit from treatment with chloroquine or hydroxychloroquine. Researchers also are investigating the reported benefits of methotrexate, azathioprine and cyclosporine in altering the lesions that threaten critical areas of the body, such as the central nervous system or other internal organs





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