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Sarcoidosis
COMMONLY by
USC Department of Medicine
LAC+ Dedication To my patients, from all walks of life and all corners of the world, who continue to reach me by asking questions. Sarcoidosis
COMMONLY Contents
INTRODUCTION More than a century
ago, Jonathan Hutchinson, a surgeondermatologist, identified the
first case of sarcoidosis at King's college, Sarcoidosis is common
in Scandinavian countries, the The cause of sarcoidosis is not known. No infective or inflammatory agent has been consistently isolated from patients with sarcoidosis. Early studies that examined the role of meteorology, soil, plants, pine pollen, proximity to woods and forests, and exposure to pets and farm animals proven to be of no avail. The disease most likely represents an inflammatory response to one or many agents (bacteria, fungi, virus, chemicals) occurring in a person with either an inherited or acquired predisposition. The basic lesion in sarcoidosis is a compact, noncaseating granuloma made up of radially arranged epithelioid cells with pale nuclei, a few giant cells, and lymphocytes. Caseation is absent; occasionally, a small area of necrosis may be present. Granuloma may resolve spontaneously, leaving no scar; may persist for a long time with little or no fibrosis; or may undergo complete fibrosis, resulting in loss of tissue architecture. The first step in the development of sarcoidosis involves the interaction of an unknown antigen or antigens and alveolar macrophages bearing increased expression of major histocompatibility complex (MHC) class II molecules. These macrophages engulf, process, and present the putative antigen(s) to T-lymphocyte cells of Th-l type. These activated T-cells release a number of cytokines, including interleukin- 2, monocyte chemotactic factor, macrophage migration inhibition factor, and leukocyte inhibitory factor. Interleukin- 2 activates and expands various clones of T-lymphocytes; monocyte chemotactic factor attracts monocytes from blood into the lungs; macrophage migration inhibitory factor influences the trapped monocytes that are ready to transform into epithelioid cells and modulate the formation of a granuloma. The granuloma formation and associated helper (CD4+) T-lymphocyte alveolitis may lead to substantial lung injury. At this time, when the lung is the site of tremendous outpouring of lymphocytes, the peripheral circulation shows a CD4+ T-cell reduction resulting in depression of cutaneous delayed hypersensitivity reactions. Simultaneously, the B-cell function is increased. It is manifested by hyperglobulinemia; increased antibodies to Epstein-Barr, herpes simplex, and other viruses, and the presence of circulating immune complexes. Activated macrophages manufacture and release a number of chemicals mediators, including fibronectin, cytokines, and growth factors responsible for causing fibrosis. Untreated sarcoidosis eventually subsides in most of the patients, but it may worsen in others. The outlook is better in patients with erythema nodosum. The patients who experience spontaneous remission rarely relapse. If pulmonary infiltration persists for more than 2 years, it is unlikely to remit without therapy. Devastating complications are due to the irreversible scarring of the lungs, the heart, the eyes, the kidneys, and the neuro-muscular system. The prognosis is poor in American blacks, particularly women, and especially those who at the time of the initial discovery have pulmonary infiltration and disease involving more than three organ systems. Corticosteroids are effective drug against sarcoidosis and its complications. Other drugs, such as chloroquine, hydroxychloroquine, methotrexate, azathioprine, pentoxyphylline, and thalidomide have been used with varying success. These drugs are of particular value in patients who cannot tolerate corticosteroids or their side effects. Lung or heart transplantation may be necessary in severe pulmonary or heart failure. QUESTIONS AND ANSWERS The Disease 1. What is sarcoidosis? Sarcoidosis is an inflammation in which lymphocytes, a type of blood cell, become overactive. These overactive lymphocytes release chemical substances which cause granulomas (a collection of inflammatory cells) in various organs of the body. Although sarcoidosis is a multisystem disorder, it affects the lungs 90% of the time, making it primarily a lung disease. 2. What does the name "sarcoidosis" mean? The name Sarcoid comes from the Greek word sarko meaning "flesh". The OID is also from the Greek and means "like". So, sarcoidosis means flesh-like or fleshy, referring to the small skin tumors that can develop. It is pronounced SAR-COY-DOSIS. 3. What causes sarcoidosis? So far, research has shown that sarcoidosis is not caused by any known bacteria, mold or dust particles. Nor is it due to any gases or fumes. Some researchers believe that sarcoidosis is caused by a virus, but the nature of such an agent remains unclear. 4. How do I know if I have sarcoidosis? Many patients do not have any symptoms, but some have difficulty in breathing. Others have dry cough; still others may have pain in the chest, tiredness, lethargy and listlessness. Sarcoidosis may also cause fever and weight loss. Whether you have symptoms or not, your chest x-ray will almost always be abnormal. 5. What organs are affected by sarcoidosis? The lungs are the most commonly affected organs by far, but the disease can also involve the eyes, skin, lymph glands, bones and joints, heart, nervous system and other internal organs. For this reason sarcoidosis is classified as a multisystem disorder. 6. What is the effect of the disease on lungs? Shortness of breath, cough, chest pain and tightness are the most common complaints of sarcoidosis of the lungs. Severe scarring (fibrosis) of the lungs can cause respiratory failure. 7. What is the effect of the disease on eyes? Burning, itching, red, light sensitive eves are the usual symptoms. If sarcoidosis of the eyes is not recognized and properly treated, blindness may occur. 8. What is the effect of sarcoidosis on skin? Reddish-blue patches, rash, nodules and small growths are features of skin sarcoidosis. Skin nodules are neither itchy nor painful. They do not weep fluid. In some cases, skin tumors or growths can become scarred and ugly. 9. What is the effect of sarcoidosis on lymph glands? Often there are no symptoms to this form of sarcoidosis, or at the most, glands in the neck, axillae and groin may become enlarged and swollen. 10. What is the effect of the disease on bones and joints? Pain in joints is usually the only symptom and often there is no pain. Unlike some forms of arthritis, bone and joint sarcoidosis does not cause crippling of any kind. 11. What is the effect of the disease on the heart? Irregular heart rhythm and episodes of dizziness are some of the major symptoms, but it is possible to be symptom free. When extensive thickening of the heart muscle with granulomas and scarring occur, sudden death can result. 12. What is the effect on the nervous system? Tingling, numbness, paralysis of the face, usually on one side, are common symptoms. Sudden onset of facial paralysis is temporary and the patient recovers full use of muscles over a period of time. Sarcoidosis of the nervous system can also result in seizures, paralysis of limbs, difficulty in swallowing, dizziness and vertigo. 13. How do I know if my sarcoidosis is serious? If your lungs have been badly scarred, and if your sarcoidosis involves the heart, eyes or central nervous system, then most likely your disease is complicated and you will need treatment by a sarcoidosis specialist. If, however, sarcoidosis has only affected the glands in your lungs, it is probable that your disease will remain harmless. 14. Is the disease contagious? There is no evidence that sarcoidosis is contagious. 15. Is my skin rash contagious? Not at all. In some patients the skin rash of sarcoidosis may look unsightly, but there is no need to worry about infecting another person. 16. Is sarcoidosis a form of lung cancer? No. Sarcoidosis is not related to lung cancer. 17. Is it related to or a form of AIDS? No. Sarcoidosis is not related to Acquired Immune Deficiency Syndrome (AIDS) or any sexually transmitted or intravenous drug use disorder. 18. Is sarcoidosis a form of Hodgkin's disease? When you see your doctor or other medical personnel for your disease, you might hear the words "Hodgkin's disease" mentioned. Remember, sarcoidosis may look like Hodgkin's disease, but it is not related to Hodgkin's disease. 19. Does sarcoidosis cause diabetes? No. Sarcoidosis does not cause diabetes, but prednisone, used for treating sarcoidosis, may cause diabetes in those patients who are predisposed to develop diabetes. 20. Is sarcoidosis an allergic disease? Sarcoidosis is not caused by any known allergic substances including pollens, grasses, fish, mites, or other animal and vegetable proteins. 21. Can sarcoidosis develop into asthma? Asthma is an allergic lung disease. Some patients with sarcoidosis may have allergies that cause asthma, but sarcoidosis does not develop into asthma. 22. Can sarcoidosis develop into emphysema? Although sarcoidosis causes lung destruction, it does not cause emphysema. 23. Could my sudden shortness of breath prove fatal? Usually, not. Shortness of breath in sarcoidosis develops over a period of time. If you have a sudden attack of shortness of breath it is probably not due to your sarcoidosis. For sudden shortness of breath, see your doctor. 24. Sometimes I feel tired. Does sarcoidosis cause fatigue? Patients with badly scarred lungs, heart or muscle involvement complain of tiredness, fatigue or lethargy due to lack of oxygen and increased work of breathing. Sarcoidosis may also cause symptoms of Chronic Fatigue Syndrome (CFS). 25. How long does it take to recover from sarcoidosis? If your sarcoidosis appears suddenly, in many cases overnight, and you have large, tender, red, bumps on your legs (erythema nodosum), then you can expect to be free of disease within 12 to 18 months. If your illness develops gradually, over many months or years, it may last much longer, anywhere from several months to your entire lifetime. 26. Can you tell me about congestive cardiomyopathy brought on by long term pulmonary involvement? How is it treated? Long term pulmonary involvement (fibrosis/scarring) causes failure of the right side of the heart called cor pulmonale. It is due to the lack of oxygen. You need to be on oxygen if your oxygen is low. Prednisone, chloroquine on hydroxychloroquine and methotrexate are the drugs used to treat sarcoidosis of the heart muscle. 27. In talking to several women who have sarcoidosis, I discovered some of us have severe back pain, in the muscle along the bra line and then up the middle, It is more severe at some times than others. Can you shed any light as to the cause? Back pain may be due to enlargement of the glands (hilar) in the chest, muscle spasm, arthritis in the spine involvement of the nerves supplying the torso or even osteoporosis related to prednisone. In some cases pain in the chest may be due to the involvement of the lung lining or pleura. Thus, persistent back pain requires careful evaluation. 28. If sarcoidosis is a multi-system disease, what is the worse case possible? When sarcoidosis involves the heart, eyes, brain and kidneys the outlook is bad. 29. There isn't much known about granulomatous nephritis. What if any relation does it have with sarcoidosis? How do you know the difference between the two diseases? If a patient with sarcoidosis develops granuloma in the kidney, the renal inflammation then most likely is due to sarcoidosis. On the other hand, if kidneys are the only organs that show granulomas, then other causes need to be excluded. 30. Has any geologic element or mineral been implicated as a possible agent causing sarcoidosis? Multisystem sarcoidosis is not caused by any element, but granulomatous disease may be caused by beryllium in the lungs and skin. The disease is called berylliosis. 31. Could "red and watery eyes" be a symptom of diseases other than those due to allergy? "Red and watery" eyes particularly with light sensitivity may be due to sarcoidosis, autoimmune disease, or local eye inflammation or tumor. 32. Are there areas
in the Sarcoidosis is common in the south and southeast; a lot of research is being done to find out the cause and distribution of sarcoidosis. 33. My brother has neuro sarcoidosis. What is it and how is it caused? Sarcoidosis of the central nervous system occurs in about 10 percent of the patients. Any part of the nervous system including the brain, meningitis, nerves and spinal cord may be damaged. 34. What is the relationship between Raynaud’ s syndrome and sarcoidosis? There is no definite relationship between Raynaud’s syndrome and sarcoidosis. Raynaud’s phenomenon or sensitivity to cold occurs in patients with scleroderma and other autoimmune diseases. 35. Can sarcoidosis cause progressive arthritis? Joints are infrequently affected in sarcoidosis; severe arthritis is rare. 36. Do patients with sarcoidosis have left arm pain? Not specifically, but chest pain may occur in patients who have enlarged hilar or mediastinal glands and in patients with sarcoidosis of peripheral nerves. 37. Are other rheumatological diseases associated with sarcoidosis? Not commonly. There are a few patients who may have sarcoidosis associated with rheumatoid arthritis, scleroderma or lupus erythematosus. 38. Are some countries or populations more prone to sarcoidosis? Sarcoidosis is very
prevalent in Scandinavian countries. The incidence there is about
600 per 100,000 of population; whereas, in 39. Is it possible that sarcoidosis will go away and never return? Yes, in patients with bilateral hilar adenopathy and erythema nodosum, sarcoidosis often goes away without any treatment. 40. Is it possible to be in remission and have no symptoms but still have low pulmonary test reading? Yes, it is possible to have mildly impaired lung function with no evidence of active disease. This is due to residual scarring of the lungs. 41. When I had a biopsy done, the specimen showed granulomas. What does it mean? I received injuries about 20 years ago. The scars now look like keloids. Granuloma is a lesion consistent with sarcoidosis. When sarcoidosis becomes active, old injuries and scars light up, become tender and firm, and on biopsy show granulomas. 42. When the "eye lesions" flare-up, what actually causes so much pain? Inflammation of the conjunction, cornea and uveal tract cause redness, pain, and light sensitivity. 43. What is the cause of the constant and persistent cough? It reminds me of my uncle who had tuberculosis. Cough is related to inflammation of the airway or bronchi. It has no relationship to tuberculosis. 44. Is it common for sarcoidosis to first appear in the lungs? Yes. Sarcoidosis commonly involves the lungs. In more than 90% of the patients chest x-ray film is abnormal. 45. Does sarcoidosis cause a lot of anxiety and insomnia? Any illness, particularly in individuals who were previously healthy, can cause anxiety and insomnia. Furthermore, anxiety may be related to lack of information about the disease. Prednisone can cause anxiety, insomnia, and irritability. 46. To what do you attribute the high number of African Americans with sarcoidosis and the fact that their disease is usually more severe than other ethnic groups? We do not know the reason. Hypertension and diabetes are other diseases that are progressive in African-Americans patients. Perhaps, yet unknown genetic or hereditary factors play a role. 47. Does sarcoidosis affect person's sleeping pattern? Only if the patient has enlarged tonsils and upper airway granulomas. In a sarcoidosis patient the presence of obesity might be the cause of sleep apnea and related breathing abnormalities. Rarely, hypothalamic involvement may cause sleep disturbances. Sleeping pattern may also be changed in CFS associated with sarcoidosis. 48. How often is sarcoidosis found in children? Sarcoidosis is rare in children. Only 2 to 3% of sarcoidosis are below the age of 10 years. 49. How does one know the stage of sarcoidosis? These stages are based on the appearance of a chest roentgenogram. 50. My father died of miliary tuberculosis. My mother had lupus; my brother had tuberculosis. I have sarcoidosis. Are these diseases related? Tuberculosis, sarcoidosis, and lupus erythematosus are not causally related. However, occasionally, a patient may develop two or all three diseases. 51. What part does stress play in relapse of sarcoidosis occurrence? Stress does not cause sarcoidosis, bur stress can increase the misery caused by the disease. 52. How does sarcoidosis affect the mental attitude of the patient? Any chronic illness will have an effect on mental attitude. Individuals become frustrated, tired and depressed. 53. Is chronic low grade fever (99.4) typical of sarcoidosis? Should any medicines be taken to reduce fever? Any type of fever is unusual in sarcoidosis. Fever may occur in sarcoidosis along with night sweats, chills and high fevers. But, every attempt should be made to exclude infections and lymphoma that commonly cause fever. 54. Is the reappearance of nodules or bumps on arms, ears, and legs a sign of sarcoidosis recurrence or flare up? Reappearance of skin lesions indicates flare up of sarcoidosis. 55. If a person is in remission, how often should he get a lung function study and chest x-ray film? Once a year. 56. How often should bone density be checked? If you are on high dose prednisone, check bone density frequently, say every 3-6 months. 57. Can joint pain and swelling last for an indefinite period? Yes, in a small number of patients mild joint pains, muscle aches, and stiffness may last for a long time. Deforming joint disease is uncommon in sarcoidosis. 58. Why is sarcoidosis found more frequently in African-Americans? The cause is not known. Perhaps, as yet unknown genetic factor playa role. In African-American patients the disease tends to be chronic and responds poorly to treatment. 59. Can you have sarcoidosis in other organs but the chest x-ray may be normal? Yes, in ten percent of the sarcoidosis patients chest x-ray film may be normal. These patients may have skin, heart, brain, bones, or joint disease. It is also possible to have sarcoidosis in the lung without it showing up on your chest x-ray film. In such cases a CT examination of the chest is indicated. 60. Is lung collapse a common occurrence in sarcoidosis? Lung collapse or pneumothorax occurs in less than 2% of the patients. These patients usually have advanced lung scarring. The Patient 61. Who gets sarcoidosis? Anyone can get sarcoidosis. The disease occurs throughout the world. Although it is more frequent in some parts of the world and less common in others, no race, sex or age group is excluded from the disease. 62. Why do we get sarcoidosis? We do not know why some people get sarcoidosis and others do not. The best that can be said about sarcoidosis at our present stage of knowledge is that when a susceptible host meets an agent which enters the body, most likely by inhalation, sarcoidosis results. 63. Are there any other ways I can help myself? You should follow sensible health measures. Do not smoke and do not drink excessively. Avoid drugs or chemicals injurious to your liver. Also, avoid exposure to toxic inhalants, dust, fumes, gases and chemicals that can injure your lungs. 64. Do I have to get rid of my pets? No. You do not have to get rid of cats, dogs, horses or other pets if you have sarcoidosis. 65. Does the disease run in families? Will my children get it? There are a few families where sarcoidosis has affected more than one member of the family. In general, you need not worry about transmitting the disease to your children. However, much research is still being done on this topic. 66. Will sarcoidosis affect my life style? Most of the patients with sarcoidosis lead normal lives. They carry on usual social, intellectual, artistic and athletic activities. Sarcoidosis, unless severe and progressive, will not interfere with your life style, nor should it interfere with the achievement of your goals. 67. Will sarcoidosis affect my pregnancy? Sarcoidosis should not interfere with pregnancy. It does not affect the unborn baby. Many women with sarcoidosis improve during pregnancy because their bodies are producing more corticosteroids. However, your doctor will check your chest x-ray three months after delivery to monitor possible recurrence of the disease. 68. Do I have to observe dietary precautions? About one in ten patients with sarcoidosis has high blood calcium levels. If your calcium level is high. avoid calcium rich substances such as milk, cheese, and any vitamins containing calcium. Also, avoid direct sunshine and vitamin D if your calcium level is high. 69. Should I take vitamin supplements? Be careful about taking calcium supplements and vitamin D if you have sarcoidosis, but other vitamins are harmless. Vitamin therapy is not indicated in the treatment of sarcoidosis, but unless you are taking excessively high does of any vitamins, you can continue your supplements. Do check with your doctor, however, before continuing with very high doses of vitamins. 70. Should I restrict my exercise? If your lungs cannot keep up with you and you feel out of breath, you should stop and rest. But do not restrict your activities just because you have sarcoidosis. 71. Will smoking affect my disease? Smoking does not cause sarcoidosis, but if you have lung disease due to sarcoidosis, then smoking may worsen your breathlessness by adding new injury to your breathing tubes in the form of bronchitis or emphysema. You should be aware that any lung disease can be further compromised by smoking. If you smoke, and you have sarcoidosis, this is a good time to help your lungs by quitting your habit. 72. Will drinking affect my disease? Moderate social drinking has no effect on sarcoidosis. 73. Will having sarcoidosis affect my sex life? Only if you have sarcoidosis of the nervous or endocrine system, might you experience impotence. This feature is rare. 74. Can I travel by air? Will It have any affect on my lungs? Patients with mild lung disease (and that describes most sarcoidosis patients) have no problem. Only those patients who have extensive fibrosis of the lungs may need supplemental oxygen. In that case, your doctor will measure your oxygen content and advise accordingly. 75. Is it true that prednisone does not change the course of the progression of the disease, it only suppresses symptoms? Prednisone suppresses granulomatous inflammation due to sarcoidosis. In some patients, sarcoidosis subsides while other patients might need prednisone for a long time. 76. Have any natural herbs or diet systems been found effective? Not to my knowledge. Treatment 77. What is the treatment for a patient who has enlarged lymph nodes and involvement of the lungs, spleen, and liver. The patient also has diabetes mellitus? Although prednisone would be an ideal drug, in this case the presence of diabetes mellitus is contraindicated. Hydroxychloroquine and methotrexate should be tried. 78. What is the best treatment for neuro sarcoidosis? At least 10% of all patients with sarcoidosis have neuro sarcoidosis. Prednisone, hydroxychloroquine, methotrexate, azathioprine, cyclophosphamide and radiation have been tried in varying dosages. 79. How long should prednisone be taken? Are there any tests to check on toxic levels? As long as it is needed to control effects of sarcoidosis. There is no blood test to assess toxic levels of steroids. 80. How does one know if the adrenal glands are still producing corticosteroids? By measuring blood cortisol levels and performing a challenge test. 81. If my calcium levels are in the normal range and I'm taking prednisone, should I be taking calcium? If your nutrition is inadequate and if you are a woman in menopausal years, you should take a calcium supplement. 82. Is plastic surgery the right treatment for my skin lesions? The most troublesome skin lesions are slowly developing reddish-blue patches (plaques) and scarred areas that occur on the face. Most of these rashes can be brought under control by corticosteroids and chloroquine, and methotrexate. Plastic surgery is rarely needed. The risk is that some sarcoidosis patients will develop raised, disfiguring scars (keloids) after surgery. You will need to discuss the pros and cons of plastic surgery with a sarcoidosis specialist before undergoing the surgery. 83. What are the side effects of corticosteroids? Corticosteroids have many side effects; some are disabling. Your doctor will discuss the effect of these drugs. Common side effects include excessive weight gain, acne, diabetes in susceptible people, high blood pressure, glaucoma, cataracts, thinning of the bone (osteoporosis) and psychological symptoms. 84. What are the side effects of the other drugs? Chloroquine does not have the same side effects as corticosteroids. However, if given for a long period of time it may cause damage to the eyes. Hydroxychloroquine does not have many side effects. Immunosuppressive drugs have more serious side effects such as increased susceptibility to infections, anemia, and suppression of the body's ability to fight disease. 85. I will start chemotherapy and radiation for uterine cancer. Will this affect my active pulmonary sarcoidosis? How? Chemotherapy, particularly methotrexate, chlorambucil and azathioprine are used in treating sarcoidosis patients when prednisone fails to control the disease or produces too many side effects. It is conceivable that your sarcoidosis may indeed improve during the chemotherapeutic treatment. 86. When I'm off steroids, my doctor tells me that I could take up to 12 Advil per day. Is the drug of any value in treating sarcoid? Advil is good for pain. It is a nonspecific analgesic and anti-inflammatory drug used in controlling joint pains. It is not used to treat sarcoidosis. 87. Has acupuncture any therapeutic value in managing sarcoidosis? Acupuncture does not cure sarcoidosis. If a patient with sarcoidosis has muscle aches and pains, acupuncture may be of help. 88. Is deflazacort being used in this country? Besides being "bone sparing", is deflazacort better than prednisone? Prednisone, cortisone, triamcinolone, hydrocortisone and deflazacort are all corticosteroids. 89. Is hypertension a side effect of long term therapy of sarcoidosis with prednisone and Imuran? Prednisone causes salt retention and may cause hypertension. Imuran does not cause an increase in blood pressure. 90. Do corticosteroids cause sterility and liver damage? Corticosteroids cause weight gain, acne, osteoporosis, hypertension, diabetes mellitus in susceptible individuals, depression and suicidal tendencies, cataract, skin bruising, and infections. There are many other side effects and the patients should always discuss with his doctor. Prednisone does not cause either sterility or liver damage. 91. What should be done to help fatigue of sarcoidosis? Fatigue in sarcoidosis is common. It is perhaps related to its cause; some patients respond to prednisone and hydroxychloroquine. 92. Would physical therapy be helpful in treating joint inflammation and muscle involvement? Yes, physical therapy along with analgesics and corticosteroids is very helpful in managing joint involvement. 93. What is the best treatment for sarcoidosis of the bones and bone marrow? Prednisone with hydroxychloroquine is an effective treatment. 94. If a person has been on prednisone and azathioprine for over six months and no improvement has been made, what other alternatives are there? First, the physician will have to reevaluate the nature of sarcoidosis. If the lungs are completely scarred no medicine will be effective. If the disease is active, explore the possibility of giving hydroxychloroquine 200 mg BID. 95. Are there any preventative measures that may be taken to avoid seasonal relapses? Sarcoidosis, unlike hay fever, asthma and other allergic diseases, has no definite seasonal exacerbations. 96. What treatment is used to prevent sarcoidosis attacking the transplanted organ? There is no known drug that will prevent sarcoidosis. If the disease appears in the transplanted organ, the treatment includes prednisone, hydroxychloroquine, and immunosuppressive drugs. 97. Is childhood sarcoidosis treated differently from adult sarcoidosis? No, the treatment is more or less the same. However, immunosuppressive drugs are usually avoided in children. Diagnostic Tests 98. What is a gallium lung scan? The test is done by injecting a radioactive substance gallium-67 into one of your veins. The gallium then is picked up by the organs affected by sarcoidosis and other inflammations. Two days after, the body is scanned and pictures are obtained to see the extent and severity of sarcoidosis. The test is not routinely performed in every patient. Discuss this with your doctor. 99. What is bronchoalveolar lavage? A bronchoscope is a long, narrow tube with a light at the end. It is used to examine the breathing tubes and the lungs. It is also used to collect fluid from the lungs. This fluid is examined for various cells and substances that reflect the inflammation and immune changes in the lungs. This process is referred to as bronchoalveolar lavage (BAL). 100. What is a lung biopsy? This procedure consists of removing a tiny piece of the lung tissue in order to examine it under the microscope. This is the single most important test in correctly diagnosing your lung disease. 101. How often should a sarcoidosis patient have a TB test and a Gallium Scan? Gallium is performed to study the extent and activity of sarcoidosis. It may be used to assess the response of the disease to treatment. A tuberculin (TB) test is performed initially when the sarcoidosis patient is investigated in order to exclude tuberculosis. 102. I have never had a gallium scan, should I? No, not if you do not have any symptoms and your physician does not feel a need for the test. 103. When is a mediastinoscopy indicated? If a patient has enlarged glands in the mediastinum and the chest, and the diagnosis has not been established by transbronchial lung biopsy, then a mediastinoscopy is indicated. 104. What tests are there for predicting disease activity in sarcoidosis? Chest x-ray film, lung function tests, serum angiotensin converting enzyme, serum calcium and Gallium-67 scan and lymphocytes in bronchoalveolar lavage are some of the tests used to assess activity. 105. How useful is the serum angiotensin converting enzyme (SACE) test? Serum ACE level is high in about 60% of the patients with active sarcoidosis. ACE level comes down when the disease undergoes remission or responds to treatment. There are other conditions that also may have high ACE levels including miliary tuberculosis, leprosy, coccidioidomycosis and lymphoma. 106. What is the single most specific test to diagnose sarcoidosis? The best way to diagnose sarcoidosis is to obtain a tissue biopsy and demonstrate the presence of noncaseating granulomas, excluding any other causes of the granulomatous reaction including tuberculosis, histoplasmosis, coccidioidomycosis, and berylliosis. 107. If a person does not have sarcoidosis and is injected with the Kveim antigen, is there any concern that this person could later develop sarcoidosis? No. 108. Is Kveim-Siltzbach test FDA approved? In this day and age of AIDS is the test safe? Kveim-Siltzbach is not approved by the FDA; the test is safe, however. 109. What medicines are used for treating of sarcoidosis? The most commonly used drugs are corticosteroids (cortisone, prednisone, methylprednisolone). Many physicians use chloroquine, hydroxychloroquine (used in the treatment of malaria) and immunosuppressive drugs (methotrexate, azathioprine, cyclophosphamide, chlorambucil). Many of these drugs are not approved by the FDA in the treatment of sarcoidosis. 110. How do I know if my disease warrants corticosteroids (prednisone, cortisone) treatment? Your physician in consultation with a sarcoidosis specialist will determine the extent and severity of sarcoidosis and advise accordingly. In general, severe shortness of breath, irregular heart rate, red eyes and nervous system involvement warrant treatment with corticosteroids. 111. What is a High Resolution Computerized Tomography (HRCT). HRCT is a special radiological test that uses a computer program to find severity and extent of lung disease. 112. Are HRCT and CT examinations the same? No, HRCT is commonly
used for sarcoidosis, interstitial lung disease, emphysema, and
bronchiectasis. 113. What is thoracoscopic lung biopsy? It is a procedure to obtain a lung biopsy specimen without opening up the chest wall The biopsy is obtained through three tiny holes in the chest. It is less invasive than an open lung biopsy. Miscellaneous 114. If a sarcoid patient is traveling overseas, what inoculations should he take? There are no special inoculations for sarcoidosis patients. They need the same inoculations as other individuals. 115. If I travel to Europe, Asia, Canada or any other country, would I be able to find a sarcoidosis specialist in case of emergency? Yes. For the address,
write to your local sarcoidosis support group. There are many such
support groups that exist in the 116. Who is the best physician to take care of my illness? Sarcoidosis is best controlled by a doctor whose special interest is sarcoidosis. Since the lungs are the most frequently affected organs, a lung specialist is often the physician who treats sarcoidosis patients. There are many sarcoidosis specialists, most of them at major medical schools and research centers. 117. Where can I read more about sarcoidosis? Your local chapter
of the American Lung Association and the GLOSSARY Angiotensin Converting Enzyme (ACE): Level of this enzyme is increased in the blood of patients with sarcoidosis. The test supports the diagnosis of sarcoidosis. Acid-fast staining: A method for recognizing the bacteria that causes tuberculosis (Mycobacterium tuberculosis). AIDS: Acquired immunodeficiency syndrome Acute illness: Illness that occurs suddenly over a short time. Adrenal Glands: Two small glands in body that make steroid hormones. Alveolar macrophage: A type of cell that is involved in lung defenses. Alveolus: Sac-like structure of the lung where oxygen exchange occurs. Alveoli is pleural. Anergy: Loss of skin reactivity to an antigen. Anesthesiologist: A doctor who specializes in anesthesia, sedation and pain control. Anorexia: Loss of appetite. Antibody: Protein complex (an immunoglobulin) that interacts with an antigen. It is produced by B-Lymphocytes and recognizes specific area of an antigen. Antigen: Substance that reacts with an antibody. It contains an area on its surface to which antibody binds. APC: Antigen presenting cell Autoimmune response: Immune system recognizes the host tissue as a foreign intruder and attacks it. B lymphocyte (B cell): Lymphocyte that produces circulating antibodies or immunoglobulins. Bacillus: A rod shaped bacterium. Bacilli and bacteria are pleural. Bacteriophage: Virus that infects bacteria. Bell's Palsy: Paralysis of the seventh cranial or facial nerve. Benign: Harmless, not cancerous. Beryllium: A metal that causes berylliosis, a lung disease that resembles sarcoidosis. Biopsy: Sampling a small portion of body tissue to find out whether tissue is benign, infectious or cancerous. Bronchoscopy: Insertion of a thin tubular instrument (bronchoscope) into the lung for sampling fluid and tissue specimens. Bronchoalveolar lavage (BAL): Fluid obtained from the lung by bronchoscopy. Cardiomyopathy: Disease of heart muscle causing heart failure. Caseation: Tissue necrosis (dead tissue) that resemble cheese, occurs in tuberculosis. CBC: Complete blood count. Normal red cell counts, 4.2 to 5.9 million/mm3. Normal white cell counts, 4,800 to 10,800/mm3. Normal platelet count, 200,000 to 350,000/mm3. Cell-mediated immunity (CMI): Acquired immunity as a result of T-lymphocytes and activated macro phages. Chemokines: Proteins produced by many human cells including endothelial cells, macrophages and cells of the immune system. Chemotherapy: Using drugs that act by altering immune mechanisms and cell growth. These drugs are used to treat lymphoma, leukemia and cancers. Cirrhosis: Chronic liver disease that interferes with normal liver function. Conjunctivitis: Inflammation of the membrane of eyes and eyelids. CT or CAT Scan (Computed Tomography Scan): A method of imaging in which a computer is used to reconstruct the anatomic features of an organ or tissue. CXR: Chest x-ray. Cytokines: Bioactive proteins produced by many human cells especially endothelial cells and macrophages. Diabetes: Also called sugar diabetes characterized by increased glucose (sugar) level in the blood. Diagnosis: The cause of an illness. Dyspnea: Shortness of breath Edema: Excessive fluid in tissues. Embolization: Procedure by which abnormal blood vessels are blocked. Emphysema: Lung destruction caused mainly by smoking. Endothelial cells: Type of cells that line blood vessels and the heart. They produce various cytokines. Erythema nodosum: Red, tender bumps on the legs in some patients with acute sarcoidosis. ESR or Erythrocyte Sedimentation Rate: A measure of tissue inflammation. Normal rate: men, 1 to 13 mm/hr; woman, 1 to 20 mm/hr. Etiology: Study of factors that cause disease. Fibrosis: Scar formation or scarring. Fungi: Microbes with rigid cell walls; includes yeast and mycelial forms. Gallium Scan: Test performed to find if sarcoidosis is active and spread to many organs. Genetic: The inherited code for human structure and function. Genome: Complete set of genes of an organism. Genotype: Genetic constitution of an organism. Granuloma: A round or oval collection of inflammatory cells including lymphocytes, macrophages and neutrophils. Helper T-cells: Class of T lymphocytes that stimulate growth of specific type lymphocytes called B cells, and cytotoxic T cells, and also activates macrophages. Hematuria: Blood in urine. Hemorrhage: Bleeding. Hepatomegaly: Enlargement of the liver. Hodgkins Disease: Type of lymphoma or cancer of lymph glands. Host defenses: Mechanism by which humans keep foreign antigens from harming them. Humoral immunity: Immunoglobulin (serum antibodies) dependent defense system. Hypercalcemia: Increased calcium in blood. Hypercalciuria: Increased calcium in urine. Hypotension: Low blood pressure. Hypersplenism: Enlarged spleen that destroys red and white blood cells and platelets. Hypertension: High blood pressure. Inflammation: The process of cytokine release and phagocyte activation resulting in redness, swelling, pain and warmth at the site of injury. Interferon: Class of proteins produced by body to neutralize an endotoxin or virus attack. Three main interferons are alpha, beta, and gamma. Interleukins: Cytokines produced by monocytes and macrophages. Kveim-Siltzbach Test: Skin test for diagnosing sarcoidosis. Lesion: Destruction of tissue resulting from inflammatory challenge. Leukocyte: White blood cell. Leucopenia: Low white cell count. Lung biopsy: Procedure to remove a tiny piece of lung in order to perform microscopic examination. Lupus Pernio: Skin lesion that affects the nose and face. Lymph node: An oval structure which is the site where lymphatic vessels come together. Lymphocytes: T-cells (helper, suppressor and cytoxic) and B-cells. Lymphoma: Cancer of lymph glands. Malignant Cells/Malignancy: Cancerous cells or cancer. Macrophage: Large tissue cell that can swallow foreign particles. It develops from monocyte. Mediastinoscopy: Procedure to remove a lymph gland from the chest to make diagnosis of sarcoidosis, lymphoma, and other diseases. Monocyte: Cell in the blood that differentiates into macrophage. MRI or MR Imaging: Imaging method using magnetic resonance. Multiorgan (Multisystem) disease: Disease that affects many or all parts of the body. Necrosis: Death of tissue. Nephritis: Inflammation of kidneys. Nodule: A knot, swelling or mass. Palsy: Paralysis or weakness of a nerve. Paralysis: Loss of strength or function. Paresis: Weakness. Paresthesia: Abnormal sensations such as tingling and prickling. Phagocyte: Host cell that swallows and destroys bacteria or foreign antigen. Pleura: Membranous cavity that surrounds the lungs. Pleural effusion: The presence of fluid in the membrane cavity that surrounds the lungs. Pneumothorax: Air in the pleural cavity. It may cause the lung to collapse. Positive TB skin test: Raised, red tender area at the site of TB skin test antigen injection. Prednisone: A commonly used steroid. Sarcoid: Comes from the Greek words: "Sarko" meaning flesh; the "Oid" means "like". Sarcoidosis: Disease that causes granulomatous inflammation. Sputum: Phlegm coughed up from lungs. Splenomegaly: Enlargement of the spleen. Syndrome: Group of symptoms and signs that characterize a specific disease. T-cell: Thymus dependent lymphocyte. TH1 and TH2 cells: Type of lymphocytes. Thrombocytopenia: Low platelet count. Tumor Necrosis Factor (TNF): Cytokine produced by monocytes and macrophages. Uveitis: Inflammation of the eye. USEFUL ADDRESSES & RESOURCES
WASOG: World Association of Sarcoidosis and Other Granulomatous The World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) was formed to encourage research and information on sarcoidosis and other granulomatous disorders worldwide. Its members include investigators in the biological and medical sciences in all continents of the world. It holds conferences at frequent intervals and publishes a scientific journal, Sarcoidosis Vasculitis and Diffuse Lung Diseases, to keep the world informed of the latest news on the disorder. Emeritus President
Dr. D. Geraint James
President:
Dr. Om P Sharma Secretary:
Dr. G. Rizzato SARCOIDOSIS ASSOCIATIONS AND PATIENT SUPPORT GROUPS:
Sandra Conroy
Mrs. A. J. Cook
Ruth Jacob
Ms. Paula Polite
Andrea Wilson
Horst Bott
C. van't Hooft
Patricia McGarvey
H. Vermeulen PHYSICIANS AND SARCOIDOSIS CLINICS
Masayuki Ando, M.D.
Robert Baughman M.D.
Jean-Francois Cordier, M.D.
Ubrich Costabel, M.D.
Rohini Chowgule, M.D.
Edwardo P. Bethlem M.D., PhD
Ron DuBois M.D.
Anders Eklund, M.D.
Johan Grunewald, M.D.
S. K. Gupta M.D.
Yutaka Hosada, M.D.
Marc A. Judson, M.D.
Yash P. Kataria
Joseph Lynch III, M.D.
David Moller M.D.
Sonoko Nagai M.D.
Paola Rottoli, M.D.
Gianfranco Rrizzato M.D.
Olof Selroos, M.D.
Gianpietro Semenzto, M.D.
A. Teirstein M.D.
Violeta Vucinic M.D.
Henry Yeager, M.D. INFORMATIVE SARCOIDOSIS WEBSITES These websites include many links to other invaluable resources: WASOG: World Association of Sarcoidosis and Other Granulomatous Disorders Resource for scientific journal Sarcoidosis Vasculitis and Diffuse Lung Diseases Link: http://www.pinali.unipd.it/sarcoid/ Dr. Sharma’s Website: Link: http://www-hsc.usc.edu/~osharma/#profile Sarcoidosis Research Institute: Link: http://www.sarcoidosisresearch.org Sarcoid Connection: The Reference Library Link: http://www.sarcoidconnection.com/ SarcoidosisOnlineSites.com: Link: http://www.sarcoidosisonlinesites.com/ SarcoidCenter.com: Link: http://www.sarcoidcenter.com/
Link: http://www.nsrc-global.net/ O.P. Sharma, M.D.
Professor of Medicine, LAC+
Sarcoidosis: A Multisystem DiseaseAs Om P. Sharma, M.D., studies the lung X-ray on his wall, a face from another time and place stares intently at him. The face belongs to Jonathan Hutchinson, M.D., captured forever in a 1890 drawing by Leslie Ward, a.k.a. "Spy," who, more than a century ago, created caricatures of famous physicians for the English society magazine, Vanity Fair. Dr. Sharma, professor of Medicine
at the University of Southern California School of Medicine and
sarcoidosis specialist, has an extensive collection of "Spy" portraits
on display in his office. The caricature of Sarcoidosis, as defined in 1976 by the Seventh International Conference on Sarcoidosis, is a "multisystem granulomatous disorder of unknown etiology, most commonly affecting young adults and presenting most frequently with bilateral hilar Lymphadenopathy, pulmonary infiltration and skin or eye lesions." Three to 5 percent of sarcoidosis cases result in morbidity due to pulmonary fibrosis, cardiac arrhythmias, renal failure, neurological involvement and blindness. Although most physicians, radiologists, pathologists and immunologists encounter sarcoidosis at some point in their practice, this commonplace disease remains primarily in the pulmonary physician's domain, as 90 percent of sarcoidosis patients have lung involvement. Because sarcoidosis mimics tuberculosis, lymphoma and other diseases, Dr. Sharma often receives requests for second opinions from pulmonary physicians, as well as ophthalmologists and dermatologists, to clarify the diagnosis. Dr. Sharma became interested in sarcoidosis
during his chest medicine residency and cardio-pulmonary fellowship
at In the "At the moment, our faculty, Fellows
and researchers follow about 400 patients with sarcoidosis," Dr.
Sharma says. He has published original studies dealing with clinical,
radiologic, immunologic, physiologic and biochemical aspects of
the disease based on his experience with these patients. The disease
occurs all over the world. In fact, sarcoidosis appears most prominently
in Scandinavian countries, with an incidence rate of 600 per 100,000
population. The exact incidence rate in the In eight out of 10 patients, sarcoidosis symptoms will subside by themselves in approximately 12 to 18 months. With other patients, corticosteroids have proven beneficial in treating pulmonary sarcoidosis, as have chloroquine, immunosuppressive drugs, oxyphenbutazone and levamisole. However, if the disease progresses without treatment, the patient's lungs may be destroyed, resulting in cor pulmonale or failure of the right side of the heart. Dr. Sharma notes that patients whose lungs have been destroyed are excellent candidates for lung transplantation because 60 to 70 percent of sarcoidosis victims are between 20 and 40 years old. He adds that many of his patients with sarcoidosis also suffer calcium metabolism abnormalities due to elevated levels of blood calcium. "In 10 to 13 percent of sarcoidosis patients, this blood calcium is increased, which, if not treated, gets deposited in various organs, including the kidneys. Kidney failure can develop, and the patients may die if the calcium level is not brought down." Dr. Sharma has linked this disorder to a vitamin D metabolite produced by macrophages, which causes increased absorption of calcium from the intestine, and raised serum calcium. In addition, Dr. Sharma is conducting HL-A testing in sarcoidosis to study genetic susceptibility. The disease tends not to run in families; but, in families where sarcoidosis has occurred, the disease appears among siblings rather than siblings and parents. This indicates that sarcoidosis may be caused by environmental factors. In a related project, Dr. Sharma
and physicians from universities in Dr. Sharma conducts weekly sarcoidosis clinics in the USC Healthcare Consultation Center and at LAC-USC Medical Center, both in Los Angeles, that attract patients from all over the world, including Japan, India, and Middle Eastern, European and South American countries. A prolific author, Dr. Sharma has published over four hundred papers, two books on sarcoidosis and one book on general pulmonary disorders. "Our large numbers of patients provide us with a tremendous opportunity to study the course of sarcoidosis and the effects of various treatments." He has just finished writing a book on Hypersensitivity Pneumonitis, a group of lung diseases caused by inhalation of organic dust particles. He also has presented numerous papers on his other professional interest, the history of medicine, to the Osler Club of London, the London Harveian Society, the American Osler Society, the Cedars-Sinai Medical History Society, and the Friends of the Norris Medical Library.
His medical heroes include William
Osler, Arthur Conan Doyle and Anton Chekhov. In addition, Dr.
Sharma serves as associate editor of Lung To receive an informative brochure on sarcoidosis (the same text as above in Sarcoidosis: Commonly Asked Questions), write to:
E-mail: osharma@hsc.usc.edu Dr. Sharma’s Website: http://www-hsc.usc.edu/~osharma/#profile For an appointment to see Dr. Sharma please call: 323-442-5100 |
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