Aging Begins at 30

Sarcoidosis

The lung is most frequently involved, especially the hilar glands at the pit (hilum) in each lung where vessels and nerves enter, seen on chest X ray. Involvement of the skin, eye, liver, and lymph nodes is also common. A person's genetic makeup may influence the disease kind and outcome. People living closely and patient family are 50 times more likely to develop it than the general population. There are seasonal, and occupational clusterings (health care workers or firefighters).

The cause is unknown. Like the tuberculosis granulomas it may be an immune reaction isolating a foreign agent. The agent might be proprionobacterium (found in normal skin and in acne), a tuberculosis-like mycobacterium or herpes type 8 (Kaposi's sarcoma associated virus), in order of likelihood. The role of interleukins (IL) (chemical cell communicators) such as IL 1,2,8,10,12,15,18 and especially IL 2 and 18 and tumor necrosis factor alpha is being studied in lung wash-outs.

Sarcoidosis occurs worldwide, 15 cases per 100,000 population, affecting most ages and races, but women are slightly more susceptible than men. Nonsmokers predominate over smokers! Blacks predominate over whites in a ratio of about 14 to 1 in the United States but not in Europe.

Most patients come to their doctors aged 20 to 40, with a chest X ray showing enlarged hilar lymph nodes or with sore red shin bumps (erythema nodosum, but there are other causes). Sarcoidosis usually starts abruptly with fever, fatigue, an ill feeling, weight loss and lack of appetite. Many have cough, shortness of breath, a vague sub-breastbone discomfort and wandering joint pains. While lung-related problems are by far the most common presentation others have eye problems or facial skin rash sometimes with facial muscle palsy. A few have mumps-like enlarged parotid glands.

After ruling out similar diseases one needs a biopsy (80% have enlarged biopsiable lymph nodes) or liver and spleen enlarged in 65%. The biopsy will clinch the diagnosis. Findings outside the lung seem to vary with sex and ethnic origin. No blood findings are characteristic and the chest X ray alone is not diagnostic.

The reviewers summarize "The most satisfying therapy for the patient and physician is none." Perhaps a third will need treatment especially those with brain-, heart- or sight-threatening disease, serious lung disease or a high blood calcium. Steroids help but there is little evidence of long-term benefit except 10% improved lung function. Antimalarial drugs or immune response modifiers like methotrexate can reduce steroid dose and side effects. Drugs such as infliximab (developed to treat septic shock), suppress the TNF alpha messages that bring excess harmful inflammatory T lymphocytes, are proving helpful. Thalidomide, the notorious cause of severe congenital defects when given to pregnant women, is also helpful.

In 66% lung inflammation quietens in 2 to 5 years. Overall the outlook is good with no significant sequelae. One of my medical friends fled from Estonia in the cold war with ten companions in a cattle truck. All got sarcoidosis. He died many years later of causes unrelated to sarcoidosis. Less than 10% of patients with sarcoidosis die because of their disease. Better understanding of this sarcoid inflammation is gradually leading to better care for the patient.

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See related Patient Topics Blood/Lymphatic System, Lungs and Breathing or Sarcoidosis.

See related Provider Topics Blood/Lymphatic System, Lungs and Breathing or Sarcoidosis.

All contents copyright © 1992-2004 the Author(s) and The University of Iowa. All rights reserved.

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