The following information is taken from an Internet research paper I did for one of my school projects, and was gathered from a number of sources, many of which are listed at the end of this paper. Many of the sources contained, basically, the same information, so I tried to eliminate the repitition and only pick out most of the basic facts. I also tried to eliminate the “medicalese” wherever possible, but, in some cases it is needed, and will help to best explain the things your doctor may have told you.

This page is broken down into sections made up of a series of frequently asked questions and the facts which most accurately answer them, according to all the information I could find. Just scroll down the page or use the links below to jump to the section you wish to read.

In the event that this information does not answer your questions about sarcoidosis, try checking out the links at the bottom of the page. It is highly unlikely that I would be able to answer any of your questions in any way other than relating my experiences with the illness and its continuing treatment. For specific medical questions, always ask your physician! Several of the links below either are to support groups or are to sites which contain information about where to find support groups.

Sarcoidosis:

A Medical Mystery, Diagnosis by Elimination

What is Sarcoidosis?

 ARCOIDOSIS IS A DISEASE ABOUT WHICH LITTLE IS KNOWN. It was first identified over 100 years ago by two dermatologists working independently, Dr. Jonathan Hutchinson in England and Dr. Caesar Boeck in Norway. Sarcoidosis was originally called “Hutchinson's disease” or “Boeck's disease.” Dr. Boeck was the one who first called it sarcoidosis, from the Greek words “sark” and “oid,” meaning flesh-like. The term describes the skin eruptions that are frequently caused by the illness.
Sarcoidosis (also known as sarcoid) is one of a group of conditions known as the “Granulomatous Disorders.” It is a disease that results from inflammation of tissues of the body in which little immune cells, which are normally part of the body's defense system, act as if they need to attack an invader, and actually fight against themselves. It can appear in almost any body organ, most often appearing in the lungs or lymph nodes, but also the eyes, skin, liver and spleen.
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What causes Sarcoidosis?

 O ONE KNOWS WHAT CAUSES THE SARCOIDOSIS, and it can appear suddenly and disappear as suddenly, or it can develop gradually and go on to produce symptoms that come and go, sometimes for a lifetime. In the large majority of cases, these symptoms clear up, either with or without treatment. In the cases where they do not heal and disappear, the affected tissues tend to remain inflamed and become scarred (fibrotic). When the organ or tissue is affected, inflammatory cells (called epitheliod cells) form clumps called granulomas (“granuloma” comes from the Latin word for little grain or granule). In most cases these granulomas disappear by themselves, but in some cases continue to form, causing permanent damage, and the illness becomes chronic. As these granulomas heal they form a scar tissue which, if extensive, can affect the function of the tissue(s) involved.
Although doctors have known about the disease for more than 100 years, there is still very little known about it. The cause of the disease is not known, although a variety of both infectious and not-infectious agents have been implicated, which may cause the exaggerated cellular immune response. Some medical researchers have suggested things as divers as pine pollen, various kinds of fumes and dust, certain farm animals, and even the metal beryllium.
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Who gets Sarcoidosis?

 OCTORS DO KNOW THAT IT IS NOT CONTAGIOUS, and it is not hereditary, although there seems to be somewhat of a genetic predisposition toward the disease in certain families. Once thought to be a rare disease, doctors now know that the illness can be found in almost any medical practice in the world, and is, indeed, the most common of the fibrotic lung disorders. From data gathered, it appears to affect both sexes, all age groups and any ethnic group. It is found most commonly in patients in the age group of 20 to 40 years old, and, in the US it is found more often in African-Americans and Caucasians of northern European descent. In Europe it appears more often in Scandinavian, Irish, British and German people, and among blacks. The best estimate today is that it occurs in about 5 in 100,000 white people in the US, overall, with it occurring about equally in men and women. Among black people, it occurs more frequently, in an estimated 40 out 100,000 people, in women about twice as often as men. In Scandinavian countries it is estimated to affect as many as 64 out of 100,000 people. From available data, it appears that Sarcoidosis is uncommon in those of Asian descent and Australian Aborigines. To this point, noone has been able to establish any commonality among these various groups.
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What are the symptoms of Sarcoidosis?

 HE SYMPTOMS OF SARCOIDOSIS are many and varied, and, in fact, this often causes the disease to be misdiagnosed. In most cases, perhaps 80%, the patient does not have any symptoms, probably never knew that he or she had the disease, did not require any treatment, and the disease goes away in six months to two years. Often the first symptoms are a shortness of breath, a dry cough, or a mild chest pain. Also, the disease can show up suddenly with the appearance of a skin rash, or the symptoms may be more general, as pain in the joints (most commonly ankles), swollen lymph nodes (glands), weight loss, night sweats, fever or general fatigue and weakness. In as many as 20-30% of Sarcoidosis sufferers the eyes can be affected, and blindness can result. The difficulty here lies in the fact that there can be eye involvement with no obvious visual problems, and, therefore, people who have been diagnosed with Sarcoidosis should be checked regularly by an ophthalmologist (NOT an optician - although many opticians are well trained in detecting the symptoms of eye diseases, the seriousness of this condition should lead you to see an opthalmologist). As many as 50% of the patients with Sarcoidosis have no symptoms when the disease is diagnosed, and it may be discovered during routine physicals or check-ups, from an x-ray. Because Sarcoidosis often mimics tuberculosis and lymphoma, it is always a good idea to get a second opinion. In cases where Sarcoidosis is suspected, this is often done by the dermatologists, ophthalmologists, and pulmonary physicians themselves.
Only in a small percentage of Sarcoidosis sufferers are the heart, brain and nervous system affected. When the spleen and liver are involved, there is a substantial risk of rupture of these organs. The spleen is an organ involved in the production and maintenance of red blood cells and the production of certain circulating white blood cells. As such, it is an important part of the lymph system and the immune system, and the patient must take appropriate measures and limit the type of activities he or she engages in to prevent the chance of rupturing the spleen. If that should happen the person can survive, but special precautions must be taken for the remainder of his or her life to avoid infections of any kind.
When the skin is involved, it may appear as raised, pink or purplish areas or as painful nodules under the skin. The later usually occur on the legs, and are often associated with arthritis.
When Sarcoidosis affects the lungs, the first symptoms will most likely be a shortness of breath (dyspnea) and a cough that won't go away, or mild chest pain. When pulmonary Sarcoidosis is serious, it can develop into pulmonary fibrosis (the abnormal formation of fiber-like scar tissue in the lungs). This distorts the lung and interferes with breathing. Pulmonary involvement occurs in as many as 20-30% of patients with Sarcoidosis.
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How is Sarcoidosis diagnosed?

 HE DIAGNOSIS OF SARCOIDOSIS IS OFTEN DIFFICULT. The symptoms are so many and so varied, they often appear to be the same as a number of other diseases. Doctors usually confirm the existence of the illness in a patient by performing numerous tests and narrowing the possibilities down until Sarcoidosis is determined by exclusion. Chest x-rays can often lead the doctor to first suspect berylliosis (a disease resulting from exposure to beryllium metal), tuberculosis, hypersensitivity pneumonitis (farmer's lung disease), fungal infections, rheumatoid arthritis, rheumatic fever or cancer of the lymph nodes. All of these possibilities must be eliminated before the physician confirms the diagnosis of Sarcoidosis. Sometimes a patient's medical history, routine tests, a physical exam or a chest x-ray can lead a doctor to a preliminary diagnosis of Sarcoidosis, but other tests must be performed to confirm this. In about 9 out of 10 Sarcoidosis sufferers, there is some type of lung problem, although there may be symptoms of specific diseases related to other organs. When the lungs are not affected and other organs are involved or the symptoms are more subtle or general, the disease is often much harder to diagnose.
No single test can be relied on for a correct diagnosis of Sarcoidosis. X-rays and blood tests are usually the first procedures the doctor will order. Also, a CT Scan (Computerized Tomography, sometimes called Cat-Scan) makes it possible to see lymph nodes and scars in the lungs when regular chest x-rays are inconclusive. Among the blood tests that are usually performed on patients suspected of having Sarcoidosis is a test for Angiotensin Converting Enzyme (ACE.) level. Because the cells that make up granulomas secrete large amounts of ACE, the enzyme levels are often higher in patients with Sarcoidosis. ACE levels, however, are not always high in Sarcoidosis patients, and increased ACE levels can show up in other illnesses. Sometimes these blood tests will also show a high blood level of calcium (which can accompany Sarcoidosis), in which case the patient is advised to avoid calcium-rich foods, vitamin D, or prolonged exposure to sunlight. Also important to the diagnosis of Sarcoidosis are tissue biopsies in which tissue is taken from an inflamed area and a microscopic examination is performed to determine the presence of granulomas. Other tests may include an electrocardiogram (ECG), eye examination, urine test, neurological exam, and breathing tests.
By performing a variety of tests called pulmonary function tests (PFT), the doctor can find out how well the lungs are doing their job of expanding and exchanging oxygen and carbon dioxide with the blood. When affected, the lungs of Sarcoidosis patients cannot handle these tasks as well as they should. This is because the granulomas and the fibrosis of the lung tissue decrease the lung capacity and disturb the normal flow of gases between the lungs and the blood. The most important of these breathing tests are spirometry, lung volume, and diffusing capacity. In some cases, measurement of blood oxygen levels during an exercise test can also be done. There is, however, nothing unique about Sarcoidosis on these tests, and so other tests must also be performed. These tests are not usually part of the diagnostic process, but rather used to determine the severity of the illness.
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How is Sarcoidosis treated?

 ORTUNATELY, MANY PATIENTS WITH SARCOIDOSIS REQUIRE NO TREATMENT. Symptoms are usually not disabling and tend, in most cases, to disappear spontaneously. When this is not the case, however, the main goal is to keep the lungs or other affected organs working and to relieve symptoms. The disease is considered to be inactive once the symptoms fade. Less than 5% of cases are fatal. After many years of experience with treating the disease, corticosteroids (usually prednisone) remain the primary treatment for the inflammation and granuloma formation. Because Sarcoidosis can disappear even without therapy, however, doctors sometimes disagree on when to start the treatment, what dose to prescribe, and how long to continue the medicine. The doctor's decision will usually depend on the organ system involved and how far the inflammation has progressed. If the disease appears to be severe, especially in the lungs, eyes, heart, nervous system, spleen, or kidneys, the doctor may prescribe corticosteroids.
There is no treatment at present to reverse the fibrosis that might be present in advanced Sarcoidosis. Treatment with corticosteroids usually results in improvement, although symptoms can reappear if the medication is stopped. In the cases where treatment is needed, it may be necessary to continue the treatment for several years, as long as the disease remains active or to prevent relapse. It is important for those with Sarcoidosis to have frequent checkups so that the doctors can monitor the illness and, if needed, adjust the treatment. Corticosteroids, for example, can have serious side effects ranging anywhere from mood swings, swelling and weight gain from fluid retention, and craving for food, to high blood pressure or high blood sugar. Long-term use can affect the stomach and skin and bones, bringing on stomach pains, ulcers, acne or loss of calcium from bones. If taken in carefully prescribed low doses, however, the benefits from the corticosteroid treatment are usually far greater than the problems.
Although doctors have found that Sarcoidosis usually responds to treatment with corticosteroids, they still do not know why. They do not know what causes the disease or if it can be prevented. In most cases, with proper treatment where required, most people with Sarcoidosis can lead a perfectly normal life. You can help yourself by not smoking, and by avoiding exposure to dusts and chemicals that can harm your lungs.
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What is being done in this field?

 mong the things currently being investigated by Sarcoidosis researchers are: TitleTop




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