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Sarcoidosis

Sarcoidosis is a disease that causes inflammation of the body’s tissues and is characterized by the presence of granulomas—small areas of inflamed cells.  Sarcoidosis can attack any organ and often affects more than one.  However, more than 90 percent of patients with sarcoidosis will have pulmonary involvement. Pulmonary sarcoidosis can cause loss of lung volume and increased lung stiffness.

  • The cause of sarcoidosis remains a mystery.  Researchers have several theories including an altered immune system that gives off an exaggerated defense reaction against some event or substance perceived as a threat and/or genetic factors. One study found that the risk for sarcoidosis was increased 4.6-fold in parents and siblings of patients with the disease.
  • Sarcoidosis is often asymptomatic.  Symptoms of pulmonary sarcoidosis, when they occur, may include a dry cough, shortness of breath, raised bumps on the shins; these may be accompanied by fatigue, weakness, weight loss and unexplained fever.
  • Pulmonary sarcoidosis is found mainly in adults between the ages of 20 and 40.  Females tend to have slightly higher rates of sarcoidosis than males. Sarcoidosis appears to occur more commonly in nonsmokers than in smokers.
  • African Americans, along with Swedes and Danes, have the highest prevalence rates of sarcoidosis in the world.  In the U.S., the lifetime risk of sarcoidosis is three times higher among African Americans than whites.  Although deaths due to sarcoidosis are uncommon, the mortality rate among African Americans is more than 16 times that of whites.
  • Studies have shown that certain populations have a greater likelihood of manifestations outside the lung. African Americans are more likely to have chronic uveitis (inflammation of the eye) and lupus pernio (painful skin lesions associated with discoloration of the nose, cheeks, lips and ears). Puerto Ricans, Mexicans and Europeans tend to suffer from Erythema nodosum (tender, red bumps, usually found on the legs).
  • Heart and eye sarcoidosis appear to be more common in Japan, where the most frequent cause of death for sarcoid patients is related to heart problems.  Elsewhere, mortality is due most commonly to lung failure.
  • Diagnostic tests include chest x-rays, pulmonary function tests and special blood tests. In most cases a biopsy is necessary to fully establish the diagnosis. It has also been suggested that with the even with the mildest cases of sarcoidosis should be evaluated at regular intervals, such as annual examinations.
  • Sarcoidosis is not disabling in a majority of cases.  The condition appears only briefly and disappears on its own without treatment.  About 20 to 30 percent of people with sarcoidosis are left with some permanent lung damage and in 10 to 15 percent the disease is progressive.  Fatality occurs in fewer than one in 20.
  • Oral corticosteroids and other drugs that control inflammation represent the mainstay of sarcoidosis treatment. As a second line therapy, immunosuppressive agents may be of some benefit. Making healthy lifestyle choices, including not smoking and maintaining a healthful diet, is an important component of living with sarcoidosis. Lung transplantation can be considered as the treatment of last resort for intractable sarcoidosis unresponsive to immunotherapy.

 

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