Sarcoidosis

Sarcoidosis is a disease of unknown cause that is common in the southeastern United States - particularly here in South Carolina. The disease is characterized by multi-system involvement of organs throughout the body though disease affecting the chest organs (lungs, lymph nodes) are most common. These disease is one of granulomatous inflammation which is a type of inflammation similar to that one gets with certain infections such as tuberculosis though so far, no one has linked a particular infection with causing sarcoidosis. The disease is more common in the Southeast and within South Carolina it’s more common close to the Atlantic Ocean. The disease is also most common, and most severe, in women of African descent and least common and least severe in white males. We still do not know why the geography and racial profile matter.

Many cases of sarcoidosis are discovered by accident in patients without symptoms but who are found to have a rash or x-ray abnormality that leads to a diagnosis. Diagnosis always requires a biopsy of affected tissue which could be skin, liver, lung, or a lymph node in most cases. The x-ray images are sometimes similar to lymphoma - a cancer of the lymph nodes, so a biopsy must be done to make sure the diagnosis is sarcoidosis and not cancer or infection.

Sometimes the disease is diagnosed in a dormant or remission state and in these cases treatment is not necessarily needed though the disease can become active so follow-up with a physician experienced in evaluation and care of sarcoidosis is necessary. Because sarcoidosis can affect the heart - particularly the electrical system of the heart, patients need annual EKG testing to watch for signs that a pacemaker might be needed as these electrical disruptions can result in syncope or even sudden death from heart block. It is generally also recommended that patients with sarcoidosis have an annual eye examination to make sure there is no eye involvement in need of treatment.

When it is determined that treatment is needed to treat flare-ups of the disease and to prevent progressive loss of lung function, steroids are typically the traditional therapy used. Steroids are inexpensive and quickly effective in most cases but carry many serious side effects including aggravating or causing diabetes, cataracts, osteoporosis, psychosis, sleep disturbances, and weight gain among other issues. For this reason, we typically transition patients who are on steroids for the disease over to alternative agents to avoid most of these side effects. In recent years, most of our sarcoidosis patients who require treatment are managed with Placquenil. This is an old malaria drug that is most commonly used for its anti-inflammatory activity in diseases such as Lupus or Rheumatoid Arthritis. The dosing we use is usually 200 mg twice daily. The most common side effect is vision loss so eye examinations are required every 6 months to monitor for signs of this but vision loss is rare at this dosing schedule.

Disease activity can be monitored based on symptoms, x-rays, CT scanning, and Gallium scanning. A Gallium scan is a 3-day nuclear medicine examination that looks for and documents areas of inflammation head to toe. A negative Gallium scan or one not showing lung activity usually means that therapy can be reduced or in some cases discontinued while a positive Gallium scan indicates more therapy is needed.

Much research is being done on this disease state to try to identify causes and to evaluate new therapies for the disease. While most people manage their sarcoidosis as a more or less nuisance disease, it can be life threatening in some cases so regular medical care is necessary.

Dr. Don Elton, MD