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Sarcoidosis is an uncommon disease affecting approximately one to two people in every 10,000. The exact cause of sarcoidosis is unknown, but doctors believe it stems from a malfunction in the immune system, causing the person‘s own immune to attack normal cells. The macrophages (a specific type of white blood cell) in patients with sarcoidosis induce inflammation in lung tissue, skin and other organs (in severe cases), causing the formation of a unique type of scar, called granulomas.
The lung is the most common organ affected in sarcoidosis. The disease usually begins in the lymph glands located in the center of the chest. These lymph glands normally help protect the lungs from infection. In the early stages of sarcoidosis, these glands become enlarged. Doctors call this pattern of enlarged glands “mediastinal adenopathy.” In many cases, sarcoidosis is discovered accidentally when physicians encounter enlarged glands on a routine chest x-ray. This early sarcoidosis (stage I) does not require treatment and in goes away spontaneously in some cases.
In patients with more advanced sarcoidosis, the granulomatous inflammation spreads into the lungs themselves, causing shortness of breath, cough, and fatigue (stage II). In the most severe cases (stage III), the inflammation causes permanent scarring of the lungs that is irreversible. In 6 to 15% of cases the granulomatous inflammation spreads to other organs besides the lungs (stage IV) . Sarcoidosis can cause lumps on the face, trunk, or backs of the legs, or a purple discoloration of the nose and cheeks (called lupus pernio by physicians).
In stage IV disease, sarcoidosis can affect the eyes causing inflammation of the area around the iris or on the retina. Some patients with sarcoidosis developed arthritis, affecting the ankles, knees, and other large joints. In less than five percent (5%) of cases, sarcoidosis causes granulomatous inflammation of the membranes around the spinal cord and other parts of the nervous system.
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The tried and true method of diagnosing sarcoidosis is by analyzing a small sample of tissue underneath a microscope (i.e. a biopsy). The inflammation of the lung tissue in sarcoidosis is close to the lining of the small bronchial tubes inside the lung, which allows physicians to obtain an adequate biopsy eighty to ninety percent (80-90%) of the time without surgery by using a fiberoptic camera called a bronchoscope. If for some reason an adequate biopsy cannot be obtained by bronchoscopy, doctors can use a needle to obtain a sample from one of the swollen lymph glands, which are common in sarcoidosis. In the most challenging situations, physicians can also use a scope inserted behind the breast bone to obtain a larger sample from the swollen lymph glands (mediastinoscopy).
A pattern of swollen glands in the chest (mediastinal adenopathy) on a cat scan is characteristic of sarcoidosis. A blood test called the angiotensin converting enzyme is usually elevated in sarcodosis as well. If a patient has a combination of these two findings, a physician will have a high index of suspicion for sarcoidosis, but a certain diagnosis still requires a biopsy. |
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Early sarcoidosis (stage I) does not require treatment in most cases. In more severe cases, physicians use a class of medications called corticosteroids to decrease the formation of granulomas and prevent the spread of inflammation into the lungs and skin. In the vast majority of cases, corticosteroids are the only required treatment.
In approximately 6 to 10% of the cases, patients do not respond to corticosteroids. These cases require treatment with a stronger medication, such as tumor necrosis factor inhibitors ( i.e. remicade, enbrel ), methotrexate or Immuran. Some patients improve with these treatments and others do not, but even in those patients who fail to respond to any treatment, death from sarcoidosis is very unusual. |
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"The more serious the illness, the more important it is for you to fight back, mobilizing all your resources-spiritual, emotional, intellectual, physical."
Norman Cousins |
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