Synopsis: Information on Sarcoidosis an inflammation which produces small lumps of cells in different organs in the human body referred to as Granulomas.
Sarcoidosis often affects other parts of the body, such as the skin, eyes and liver. Less frequently, Sarcoidosis affects other parts of the body to include the spleen, nerves, heart, brain, salivary glands, tear glands, joints or bones. On rare occasion, Sarcoidosis may even affect the kidneys, breasts, thyroid gland, or reproductive organs. It nearly always involves more than a single organ, and has both active and non-active phases.
In its active phase, Sarcoidosis forms granulomas which form and grow; and symptoms may develop. There is the potential for scar tissue to form in affected organs where granulomas have developed. In the non-active phase of Sarcoidosis inflammation decreases, while granulomas stabilize in size or reduce in size. Any scarring that has been produced remains, presenting the potential to cause symptoms. The course Sarcoidosis takes varies from person to person.
In a number of people who have Sarcoidosis their symptoms are mild, and the inflammation they experience could be something that is better left on its own. Granulomas associated with Sarcoidosis might cease their growth; shrink, and any symptoms could diminish over a few years. For others, the inflammation does not go away, but it doesn't worsen either. They may have flare-ups and symptoms which require treatments on occasion. Still others experience Sarcoidosis which worsens over the years, causing permanent organ damage. The damages done involve scar tissue in the eyes, skin, organs, and on the skin. Scar tissue may affect how the organs these people have work, and while treatment can help, it does not usually affect scar tissue.
Sarcoidosis is something that does not usually cause sudden illness, and usually happens over months of time. Some of the symptoms can appear rapidly, however. These symptoms include eye symptoms, a disturbed heart rhythm, or arthritis in the ankles. More serious cases of Sarcoidosis involving vital organs can even result in death. Sarcoidosis is not; however, a form of cancer, and there is no known way to prevent it.
Sarcoidosis affects many thousands of people in the United States, and at one point in history was thought to be an uncommon condition. Knowing exactly how many people have Sarcoidosis is difficult because many people who have this condition display no symptoms. Since it was identified in the late 1860's, scientists researching Sarcoidosis have created better means of testing for it and diagnosis it, as well as methods of treating it.
Causes of Sarcoidosis
Medical science does not know what causes Sarcoidosis. Current thought is that Sarcoidosis develops when the immune system responds to things such as chemicals, viruses, bacteria or dust in the environment, or even to the body's own tissue. The immune system usually defends the body against harmful, foreign items by sending defense cells to the organs that are affected. The defense cells release chemicals which produce inflammation around the foreign items in order to isolate and destroy them. With Sarcoidosis, the inflammation does not go away, remaining and leading to the development of granulomas or lumps. Researchers have yet to identify the substance or substances that prompt the immune system to respond initially. One of the thought lines they are pursuing is that Sarcoidosis is something that develops in persons who have inherited a particular combination of genes.
Sarcoidosis is something that affects people of every race, and from all age groups. You cannot, 'catch,' Sarcoidosis from a person who has it. Sarcoidosis occurs most commonly in people of Asian, Irish, German, Scandinavian, African-American, and Puerto Rican descent who are between the ages of twenty and forty. In America, Sarcoidosis affects persons of African-American descent slightly more often and severely than Caucasians.
Studies have been completed showing that Sarcoidosis is more likely to affect certain organs in specific populations. In Japan, Sarcoidosis of the eye and heart are more common. In Northern Europe, painful skin lumps on the legs happen more often. There are also particular workers who are at greater risk of experiencing Sarcoidosis. Elementary and Secondary school teachers and Firefighters, non-smokers, Healthcare workers, and persons who have been exposed to agricultural insecticides, dust, pesticides or molds are at greater risk. Parents, children; sisters and brothers of persons with Sarcoidosis are more likely to develop it.
Signs and Symptoms of Sarcoidosis
A number of people with this condition do not experience symptoms. The condition is discovered accidentally through a chest x-ray they have received for another reason. Others have some symptoms, while yet others have several; the symptoms people experience usually depends on the organs which are affected. There are some symptoms to watch out for involving the lungs, eyes, heart, and others.
Symptoms that may be related to Sarcoidosis in association with the lungs include a dry cough which does not bring up mucus or phlegm. Wheezing and shortness of breath are other signs. You may experience pain in the center of your chest which worsens when you breathe deeply. Enlargement of the lymph nodes, commonly in the neck and chest, but at times under the chin or under the arm pits or in the groin, may be related to Sarcoidosis.
There are different skin symptoms that may be signals of Sarcoidosis as well. Painful bumps that appear on the shins or ankles which are tender, warm, and red or purple-to-red in color and somewhat raised may be a sign. They are called, 'Erythema Nodosum,' and might have an accompanying fever and swelling of the ankles and potential joint pain. These bumps will usually disappear within a few weeks to months despite going untreated. There are also a number of ulcers, bumps or rare and flat areas of discolored skin that might appear close to the eyes, nose, on the scalp or on the arms, legs or back. These may itch, are usually not painful, but can last a long time. 'Lupus Perenio,' or disfiguring skin sores, can affect the nasal passages, nose, ears, eyelids, cheeks, and fingers. These sores are usually ongoing and may return despite being treated.
Sarcoidosis presents symptoms that can affect the eyes, including sensitivity to light, dryness, burning, itching, pain, and tearing. A person may develop, 'red-eye,' or, 'floaters,' (seeing spots). Blurred vision, a reduction in color vision, or even blindness in rare cases are other symptoms of Sarcoidosis.
Heart symptoms related to Sarcoidosis include shortness of breath, wheezing, irregular heartbeat, palpitations, skipped beats, and swelling in the legs. Other symptoms involve sudden loss of consciousness, or even sudden death.
Joint symptoms of Sarcoidosis can involve joint pain, stiffness, and swelling - commonly in the feet, ankles, and hands. A person may experience painful arthritis in their ankles resulting from Erythema Nodosum, or painless arthritis that lasts for weeks or months. Muscle symptoms can include aches, muscle weakness, or a mass in a muscle.
There are bone symptoms of Sarcoidosis, including anemia resulting from granulomas which affect the bone marrow. Other symptoms may include swelling, commonly in the fingers, as well as painless holes in bones.
Liver symptoms of Sarcoidosis involve fatigue, itching, fever, an enlarged liver, or pain in the right upper portion of the abdomen, underneath the right ribs.
Salivary Gland and Parotid symptoms of Sarcoidosis can involve dryness in the throat and mouth, as well as swelling which makes the person's cheeks appear puffy.
Kidney, urinary tract, and blood symptoms of Sarcoidosis may involve confusion, increased urination, and an increased amount of calcium in either your urine or blood, which may lead to kidney stones.
There are some symptoms of the nervous system associated with Sarcoidosis; they include vision problems, numbness or weakness in a leg or arm, and headaches. A person may experience pain or weakness, or a, 'stinging needles, 'feeling in an area that is being affected by Sarcoidosis due to nerve affection. A person might also experience a, 'drooping,' of one side of their face as a result of Sarcoidosis affecting a nerve or nerves in their face. In rare cases, a person may experience a coma.
While symptoms of the Pituitary Gland are rare in relation to Sarcoidosis, they do happen. The symptoms may include vision problems, numbness or weakness in a leg or arm, or headaches. In rare cases, a person might experience a coma.
Additional symptoms of Sarcoidosis include an enlarged spleen leading to decreased platelet counts in the blood and pain in the abdomen, and a nasal obstruction or recurrent bouts of sinusitis. General symptoms of Sarcoidosis include fever, sleep problems, night sweats, fatigue, weakness, and a sense of feeling ill.
Sarcoidosis is diagnosed by taking a detailed medical history and conducting a physical examination, along with a number of diagnostic tests. There are different goals in mind; one of them is to rule out the presence of other causes of the symptoms the person is experiencing. Healthcare workers seek to identify the presence of granulomas in any of the organs in the person's body, and determine the amount of damage that has been done to any of the person's organs. Healthcare workers also determine whether or not the person needs treatment.
The person's medical history is something that healthcare workers pursue in detail in order to find information such as whether the person has a family history of Sarcoidosis, what form of work they have done, or if they have been exposed to inhaled beryllium metal or organic dust. Beryllium metal and certain organic dusts can create granulomas in the lungs that appear the same as granulomas caused by Sarcoidosis, but are signs of other conditions.
During a physical examination a doctor looks for symptoms of Sarcoidosis such as an enlarged liver or spleen, red bumps on the skin, swollen lymph nodes, or redness in the eyes. They listen to the person's heart and lungs for any abnormalities, and check for additional causes or symptoms. If a doctor suspects Sarcoidosis, they may pursue diagnostic testing.
Diagnostic testing for Sarcoidosis may involve a number of tests; there is no single specific test for the diagnosis of Sarcoidosis. Diagnosing Sarcoidosis in the nervous system or the heart, for example, is more difficult that in other parts of the body. A doctor may very well conduct a number of tests and various procedures in order to make a diagnosis of Sarcoidosis.
Chest x-rays used in diagnosing Sarcoidosis by doctors involve a staging system of 0-4. Approximately 95% of persons with Sarcoidosis have an abnormal chest x-ray. The staging system used by doctors' looks like this:
Using this staging system, the higher the stage, the worse the person's symptoms and lung function are. There are many differences between individuals, and persons with x-ray results in stages 0-3 might not present symptoms, need treatment, and could very well improve and present with a stage 0 x-ray over a period of time.
Blood testing for Sarcoidosis may reveal both the type and number of cells in your blood, and whether there are increases related to the calcium levels or changes to your kidney, liver, or bone marrow in relation to Sarcoidosis.
There are two lung tests a doctor may use in the diagnosis of Sarcoidosis. One of these tests uses a, 'Spirometer,' which measures the speed and quantity of air you can expel from your lungs after taking a deep breath. A second test measures the quantity of air you can take into your lungs. Sarcoidosis can scar the lungs, or cause them to shrink, reducing their capacity.
An, 'Electrocardiogram,' or 'EKG,' test helps to show a doctor whether or not your heart has been affected by Sarcoidosis. Another test a doctor may perform is a Pulse Oximetry, which involves attaching a small clip to your finger which can show the oxygen level in your blood.
A, 'Fiberoptic Bronchoscopy,' is more involved, and requires a doctor to insert a long, thin, tube with a light on one end through your mouth or nose and into your lungs in order to examine your airways. In order to perform a Bronchoscopy, the doctor would apply a local anesthetic. During a Bronchoscopy, a doctor may choose to pursue a, 'Bronchoalveolar Lavage.' The doctor injects a small amount of saline through the Bronchoscope and into the person's lungs, washing the lungs and helping to bring up cells and materials from the air sacs where inflammation may have developed. The fluid and cells can then be examined for signs of inflammation.
A doctor may choose to take a Biopsy, or small sample of tissue, from an organ affected by Sarcoidosis in order to do further testing and confirm the diagnosis. A person may have Sarcoidosis in more than one organ, and multiple biopsies could be needed.
Computerized Tomography Scan testing is an option a doctor may choose in order to obtain a computer-generated image of organs that have been affected by Sarcoidosis. The images obtained provide more detail and information. The images taken can, for example, give a doctor more information about how a person's lung has been affected by Sarcoidosis, or even detect Sarcoidosis in the liver.
Magnetic Resonance Scan testing is another test a doctor may order. This test can provide a doctor with images of organs that your doctor does not wish to risk performing a biopsy on; the brain for example, or the heart. It can also give the doctor images of the nerves or the spinal cord.
Gallium and Thallium Scan testing are ordered by doctors to find out if Sarcoidosis is affecting a person's heart. Both Gallium and Thallium are radioactive elements that a doctor can inject in small amounts into a vein in a person's arm, where they collect at places in the body which are inflamed. After a period of time, the person's body is scanned for radioactivity, and places where Gallium and Thallium have gathered may be signs of inflammation. Testing with these radioactive elements can give the doctor information about tissue in the body that has been affected by Sarcoidosis and the amount of damage done, but it also shows all of the inflammation in the body - even inflammation not associated with Sarcoidosis. The test does not provide a definite diagnosis of Sarcoidosis.
Positron Emission Tomography (PET) Scan testing is another test a doctor may order that uses radioactive injections. This form of testing may be more sensitive than Gallium and Thallium testing in finding areas of inflammation, and some doctors prefer to use PET scans over Gallium and Thallium tests.
Medications and Sarcoidosis
The main medication used in the treatment of Sarcoidosis is a corticosteroid known as, 'Prednisone.' Prednisone is an anti-inflammatory medication which is sometimes used in conjunction with other medications, even other corticosteroids. Prednisone nearly always relieves inflammation, and is usually prescribed for several months or for a year or more. At lower doses, Prednisone can relieve symptoms of Sarcoidosis without major side effects. There are; however, some side-effects of Prednisone that persons taking it have reported; these include:
There are other medications which can be taken to treat Sarcoidosis, and your doctor may prescribe them if you can no longer tolerate Prednisone, or if you get worse while taking it. The majority of these medications are immune suppressants which prevent your immune system from fighting viruses and bacteria, increasing the risk that you will get an infection. These medications may increase your risk of getting cancer, and may have serious side effects. Some of the medications are oral ones, while others are applied locally.
Medications that can be used in local treatment come in the form of skin creams, eye drops and medications that are inhaled. Other forms of medications include:
Hydroxychloroquine: Hydroxychloroquine is a medication used to help people with Sarcoidosis of the skin; the medication can upset a person's stomach and cause eye problems.
Methotrexate: Methotrexate is a medication that is taken either by injection or once a week and commonly takes up to six months in order to relieve symptoms of Sarcoidosis. Methotrexate has side effects that include mouth sores, nausea, decreased white cell count, liver damage, and in rare cases; a potential allergic reaction in the lungs. Pregnant women should not take Methotrexate.
Azathioprine: Azathioprine is a mediation which works in approximately half of persons with Sarcoidosis, and takes at least six months in order to relieve symptoms. It has side effects which include a reduced white cell count and nausea. Azathioprine has caused cancer in some people. Pregnant women should not take Azathioprine.
Cyclophosphamide: Cyclophosphamide is a highly toxic medication that is rarely used in the treatment of Sarcoidosis. It is only given to persons with serious forms of Sarcoidosis, such as Neurosarcoidosis. Cyclophosphamide is more likely to cause a reduced white cell count and nausea than either Azathioprine or Methotrexate, and can irritate the bladder. It may cause a bladder infection. The medication can be administered intravenously in order to lessen the side-effects; however, this does not decrease the risk of cancer. Pregnant women should not take Cyclophosphamide.
Potential New Medications
Medical science is studying medications that are used for other conditions in order to find out if they can help persons with Sarcoidosis. These medications include:
Etanercept: Etanercept is an immune suppressant that is injected underneath the skin in order to reduce the symptoms of Rheumatoid Arthritis, and can also be used to treat Ankylosing Spondylitis and Psoriasis. Studies are being done, and are suggestive that it might be useful in treating Sarcoidosis as well.
Infliximab: Infliximab is an immune suppressant which is injected into a persons arm, used in the treatment of Chrohn's Disease, Ankylosing Spondlylitis, and Rheumatoid Arthritis. Some of the studies performed show that it has helped persons with Sarcoidosis who have eye disease, Neursarcoidosis, or lupus pernio. Unfortunately, the medication has serious side-effects, but it might help improve the lung function for some persons with Sarcoidosis who have not benefited from corticosteroids.
Pentoxifylline: Pentoxifylline is another immune suppressant medication; it has both gastrointestinal and stomach side-effects. Some studies have shown that persons with Sarcoidosis in their lungs have been able to reduce the amount of Prednisone they take while on Pentoxifylline.
Thalidomide: Thalidomide is an immune suppressant that has shown effectiveness in fighting against granulomas of the skin, and scientists are studying it to see if it is effective with Sarcoidosis as well.
Tetracycline: Tetracycline is an antibiotic that is often used in the treatment of Lyme disease, some forms of Pneumonia, and Acne. There have been a few studies that are suggestive that Tetracycline might help in treating persons with Sarcoidosis in the skin.
Pregnancy and Sarcoidosis
A number of women are able to give birth to healthy children while they are being treated for Sarcoidosis. They are able to continue corticosteroid treatment during their pregnancy, although there are several medications that are not recommended while a woman is pregnant. Women who have severe forms of Sarcoidosis and are older may have difficulty getting pregnant. There is the potential that Sarcoidosis may get worse once a child has been delivered. It is important to speak with your doctor, and to receive pre and post natal care.