The Role of Inflammation in Sarcoidosis

Granulomas Form in the Body

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Sarcoidosis is a disease that can occur throughout the body but mostly affects the lungs. In sarcoidosis, inflammation produces lumps of cells (granulomas) in the body tissues. Granulomas can grow and clump together and affect how an organ in the body works. Why granulomas form is not clear, but researchers think sarcoidosis develops when the immune system responds to something in the environment.

Sarcoidosis affects men and women of all ages and ethnic backgrounds. It is most often diagnosed in adults between 20 and 40 years of age. Sarcoidosis occurs most often in Swedes, Danes, African Americans and people of Asian, Irish and Puerto Rican background. People who are more likely to develop sarcoidosis include:

  • healthcare workers
  • nonsmokers
  • elementary and secondary school teachers
  • people exposed to agricultural dust, insecticides, pesticides or mold
  • firefighters

Symptoms of Sarcoidosis

Many people who have sarcoidosis (30 to 50 percent) have no symptoms. Often, the disease is discovered by accident when these people have a chest x-ray for something else. About one-third of people with sarcoidosis have general symptoms such as:

  • fever
  • fatigue, weakness
  • weight loss or loss of appetite
  • don’t feel well (malaise)

People with sarcoidosis whose lungs are affected by the disease (90 percent of individuals) have symptoms such as:

Skin symptoms in sarcoidosis (25 percent of individuals) may include:

  • itchy lumps, ulcers or areas of discolored skin near the nose, eyes, back, arms, legs or scalp
  • painful bumps on the ankles or shins that are red or purple to red in color and slightly raised (called "erythema nodosum")
  • skin sores on or inside the nose, on the cheeks, ears, eyelids or fingers (called "lupus pernio")

Sarcoidosis can affect the eyes, heart and other organs of the body, causing additional symptoms. Sarcoidosis can also cause joint and muscle pain. The symptoms of sarcoidosis may come and go or last a long time. Some people may have only a few symptoms; others may have many problems.

Diagnosing Sarcoidosis

The diagnosis of sarcoidosis can be challenging because of the many different symptoms it can produce and because many diseases can cause the same symptoms. A detailed medical history and complete physical exam may help identify sarcoidosis. The physician will most likely conduct a variety of tests, such as chest x-ray, lung function tests, electrocardiogram (ECG) and computerized tomography (CT) scan to look for signs of sarcoidosis and help with the diagnosis. A sample of lung tissue (lung biopsy) is usually taken to look for the presence of granulomas.

Treating Sarcoidosis

Sarcoidosis varies from person to person, so treatment will be different for each patient and depends upon how he or she is experiencing the disease.

 Knowing how much of the body is affected by sarcoidosis and how active the disease is will help determine the course of treatment.

People without symptoms generally don’t need treatment. For those who have symptoms, the medication prednisone is the main treatment. Other medications, such as Rheumatrex (methotrexate) or Imuran (azathioprine), may be given along with prednisone or taken alone. Other medications may be needed depending on the symptoms, such as eye drops or heart drugs. Erythema nodosum usually goes away without treatment. Lupus pernio can be treated with creams or medications that are taken by mouth or injected in to the skin.

Sources:

"Sarcoidosis." Lung Diseases. June 2007. National Heart Lung and Blood Institute. 15 Aug 2008

Wu, Jennifer J., & Karin Rashcovsky Schiff. "Sarcoidosis." American Family Physician 70(2004): 312-322.

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