Questions and Answers - The Disease

Common questions, with answers about the disease, by the late Dr. Om P. Sharma.

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 do'sls.

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. Sarcoidosis is likely a result of continued presentation of a poorly degradable antigen. Mycobacterium tuberculosis is a strong contender for this antigen, but the organism has not been so far found in sarcoid granulomas. The molecular studies demonstrate mycobacterial deoxyribonucleic acid (DNA) in the sarcoid tissue and antibodies against it in the blood serum. These findings reinforce the possible pathogenic role of mycobacterial antigens in sarcoidosis, but the clinical utility is limited.

4. How do I know if I have sarcoidosis?

About 50% of the patients with sarcoidosis do not have any symptoms and are recognized when a chest x-ray or chest CT is obtained either as a part of routine physical examination or for evaluation for some other clinical illness. Shortness of breath is a common symptom. Some patients have troublesome dry cough and others may have pain in the chest. Tiredness, lethargy, listlessness and joint pains are common. Sarcoidosis may also cause fever and weight loss.

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. Although some organs e.g., pleura. peritoneum and adrenal glands are not frequently affected, no organ is exempt from sarcoidosis.

6. What are the symptoms of the lung involvement?

Shortness of breath, cough, chest pain and tightness are the most common complaints of sarcoidosis of the lungs. Whether you have symptoms or not, your chest x-ray will almost always be abnormal. Severe scarring (fibrosis of the lungs can cause respiratory failure.

7. What is the incidence of chest pain in sarcoidosis?

Non-specific chest pain is not uncommon in sarcoidosis. In a study of 821 sarcoidosis subjects, 19% had chest pain. The nature of chest pain not clear but it correlates with a radiological stage of the disease. The enlarged hilar nodes (Stage I can cause chest pain much like that of coronary disease. The pain is usually behind the breast bone. It can be pleuritic in nature i.e., pain worsens on taking a deep breath.

8. What are the symptoms of eye sarcoidosis?

Burning, itchy, red, light sensitive eyes are the usual symptoms. If sarcoidosis of the eyes is not recognized and properly treated, blindness may occur.

9. 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/growths can be become scarred and deformed.

10. What is the effect of sarcoidosis on lymph glands?

Often there are no symptoms to this form of sarcoidosis, but the patient may be able to feel enlarged glands in the neck, armpits, and groin. The enlarged glands usually are not tender and are painless.

11. What is the effect of the disease on bones and joints?

Sarcoid bone lesions are usually painless and discovered on bone scans. Unlike some forms of arthritis, bone and joint sarcoidosis does not generally cause crippling joint disease in the majority of individuals, but on rare occasions it has been seen. (Note: Dr. Afshars has suggested this change to Dr. Sharma`s original answer)

12. How common is sarcoidosis of the heart and what are its effects?

Sarcoidosis of the heart is a serious complication of sarcoidosis. Symptomatic cardiac sarcoidosis occurs in about 5% of sarcoidosis patients. Complete heart block or heart rate below 40 beats per minute, palpitation, irregular heart rhythm, dizziness and syncope are some of the major symptoms, but many sarcoidosis patients with heart involvement are symptom free. When extensive thickening of the heart muscle with granulomas and scarring occur, heart failure and sudden death can result.

13. What is the incidence of ventricular aneurysm?

In a necropsy study aneurysms were found in 9 out of 86 subjects. Ventricular aneurysms without evidence of coronary artery disease have been described in sarcoidosis.

14. How is sarcoidosis of the heart diagnosed?

Coronary angiography is usually normal, but when granulomatous vasculitis is present then the evidence of thrombosis and aneurysm can be observed. MRI has a sensitivity of 100% and specificity of 78% for the diagnosis of sarcoidosis. Late mycocardial enhancement is typical, but various patterns of injury have been described.

15. Can abdominal pain occur in sarcoidosis?

Abdominal pain is an unusual symptom in sarcoidosis patients. When it occurs it is attributed to pancreatitis due to hypercalcemia, as well as to enlarged liver or spleen. A few cases of peritoneal sarcoidosis have been described. A laparoscopic peritoneal-omental biopsy will show non-caseating granulomas.

16. What is the effect of sarcoidosis on the nervous system?

Tingling, numbness, and 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, muscle weakness, paralysis of limbs, difficulty in swallowing, dizziness and vertigo.

17. How do I know if my sarcoidosis is serious?

If your lungs have been badly scarred, and if your sarcoidosis involves the heart, eyes, central nervous system or kidneys, then 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.

18. Is the disease contagious?

Sarcoidosis is not contagious.

19. Is my skin rash contagious?

Skin lesions are not contagious. In some patients the skin rash of sarcoidosis may look unsightly, but there is no need to worry about infecting another person.

20. Is sarcoidosis a form of lung cancer?

Sarcoidosis is not related to lung cancer or any other type of cancer or lymphoma.

21. 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. Occasionally sarcoidosis may occur in a patient with H IV infection.

22. 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.

23. 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.

24. 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.

25. Can sarcoidosis develop into asthma?

Asthma is an allergic lung disease. Some patients with sarcoidosis may have allergies and asthma, but sarcoidosis does not develop into asthma.

26. Can sarcoidosis develop into emphysema?

Although sarcoidosis causes lung destruction, it does not cause emphysema which is caused by smoking.

27. Could my sudden shortness of breath prove fatal?

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. Lung collapse or blood clot in the lung may cause sudden shortness of breath. It is important to see your doctor promptly should you develop sudden shortness of breath.

28. Sometimes I feel tired. Can sarcoidosis cause fatigue?

Patients with badly scarred lungs, heart or muscle involvement may complain of tiredness, fatigue or lethargy. This is due to lack of oxygen and increased work of breathing. Sarcoidosis may also cause symptoms similar to Chronic Fatigue Syndrome (CFS).

29. 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.

30. What is cor-pulmonale? 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. These patients may also have pulmonary hypertension. You need to be on oxygen if your oxygen is low. Prednisone, chloroquine or hydroxychloroquine and methotrexate are the drugs used to treat chronic lung sarcoidosis.

31. 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. The pain you describe is seen frequently in overweight patients who have been on prednisone for a long time. In overweight patients gastro-esophageal reflux (GERD) can also cause such non-specific pains. In some cases pain in the chest may be due to the involvement of the lung lining or pleura. The persistent back pain requires careful evaluation.

32. In multi-system sarcoidosis, what is the worse case possible?

In pulmonary sarcoidosis progressive scarring of the lungs and respiratory failure requiring lung transplant are serious. When sarcoidosis involves the heart, eyes, brain and kidneys the disease is severe, but prompt and appropriate treatment often improves prognosis.

33. 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?

Kidneys are not commonly affected by sarcoidosis. However, if a patient with sarcoidosis develops granuloma in the kidney, the renal inflammation is likely is due to sarcoidosis. On the other hand, if kidneys are the only organs that show granulomas and the patient does not have multi-system sarcoidosis, then other causes of kidney disease need to be excluded.

34. Has any geologic element or mineral been implicated as a possible agent causing sarcoidosis?

Multi-system sarcoidosis is not caused by any element, but granulomatous disease of the lung and skin may be caused by beryllium. The disease is called berylliosis. Zirconium (used in deodorants) can cause granulomas in the skin, but it does not cause not systemic sarcoidosis.

35. Could "red and watery eyes" be a symptom of diseases other than those due to allergy?

"Red and watery" eyes particularly associated with light sensitivity is a feature of acute sarcoidosis. Similar findings may also occur in autoimmune diseases and local eye inflammation.

36. Are there areas in the US where sarcoidosis is more prominent? If the answer is yes, has any research been done to find out the reason?

Sarcoidosis is common in the south and southeast. A lot of research is being done to find out the cause and effective treatment of sarcoidosis. The National Institutes of Health strongly supports sarcoidosis research. A recent study of 59,000 black women who participated in the Black Women's Health Study, showed high incidence and prevalence of sarcoidosis amongst black women. They also had more sarcoidosis of the organs other than the lungs, Their disease required long treatment.

37. My brother has neurosarcoidosis. 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. Its cause is not known, Most of the patients respond to corticosteroids and anti-tumor necrosis factor agents.

38. What is the relationship between Raynaud's syndrome and sarcoidosis?

There is no relationship between Raynaud's syndrome and sarcoidosis, Raynaud's phenomenon or sensitivity to cold occurs in patients with scleroderma and other autoimmune diseases. Occasionally, it can occur in a sarcoid patient, but it is not a diagnostic feature.

39. Can sarcoidosis cause progressive arthritis?

Joints are infrequently affected in sarcoidosis; severe, disabling arthritis is rare.

40. Do patients with sarcoidosis have left arm pain?

Not specifically. Left sided chest pain particularly with exercise is due to coronary artery disease. Chest pain may occur in patients who have enlarged hilar or mediastinal glands and in patients with sarcoidosis who have peripheral nerve involvement. If sarcoidosis patient develops sudden severe pain, he/she needs to see a doctor to exclude pneumothorax (air in the pleural cavity) and a blood clot in the lung.

41. Are other rheumatological diseases associated with sarcoidosis?

Infrequently, sarcoidosis may coexist with rheumatoid arthritis, scleroderma or lupus erythematosus.

42. Are some countries or populations more prone to sarcoidosis?

Sarcoidosis is prevalent in Scandinavian countries. The incidence there is about 600 per 100,000 of population; whereas, in America the incidence is about 40-60/100,000. In black women sarcoidosis has an average annual incidence rate of 71/100,000.

43. 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.

44. 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.

45. 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 an inflammatory lesion and consists of epithelioid cells, lymphocytes, and giant cells. There are many causes of granuloma formation. In some cases with active sarcoidosis, old injuries and scars light up, become tender and firm, and on biopsy show sarcoid granulomas. This can happen in the patients who are prone to developing keloids.

46. When the "eye lesions" flare-up. what actually causes so much pain?

Inflammation of the conjunctiva, cornea and uveal tract causes redness, pain, and light sensitivity.

47. 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 is a common symptom and occurs in chronic bronchitis, emphysema, asthma, pneumonia, tuberculosis, heart failure and, in some patients with sarcoidosis, Tuberculosis patients may have cough, but every patient who has cough does not necessarily have tuberculosis.

48. Is it common for sarcoidosis to first appear in the lungs?

Yes, Sarcoidosis commonly involves the lungs. I n more than 90% of the patient's chest x-ray film is abnormal.

49. 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. Anxiety and insomnia are not symptoms of sarcoidosis.

50. 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, but it is related to genetic makeup. In African-Americans the liver, spleen, lymph nodes and skin are more frequently involved. Hypertension and diabetes are other diseases that are progressive and troublesome in African Americans patients. Heart and eye involvement is more common in the Japanese patients. Erythema nodosum is seen more frequently in Scandinavian patients. Research is being done to explore genetic susceptibility in sarcoidosis.

51. Does sarcoidosis affect person's sleeping pattern?

Sarcoidosis is associated with sleep apnea syndrome, particularly if the patient has enlarged tonsils and upper airway granulomas. In other patients obesity causes sleep apnea and related breathing abnormalities. Rarely, hypothalamic involvement in the brain may cause sleep disturbances. Sleeping patterns may also be changed due chronic fatigue in some patients with sarcoidosis.

52. How often is sarcoidosis found in children

Sarcoidosis is rare in children. Only 2 to 3% of sarcoidosis patients are below the age of 10 years.

53. How does one know the stage of sarcoidosis?

Stages of sarcoidosis describe the chest x-ray abnormality: Stage I disease has only enlarged hilar and mediastinal lymph nodes (these gland sit outside the lungs); Stage II disease has enlarged lymph glands as seen in Stage I disease, but it also has inflammation in the lung tissue commonly called lung infiltrates, nodules, spots or patches of the disease; Stage III has only lung infiltrates and fibrosis (scarring of the lung); Stage IV has lung destruction and advanced fibrosis.

54. 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 any two or all three diseases. In your case with such a strong family history of tuberculosis, your doctor should do all appropriate tests to make sure that you do not have tuberculosis. Occasionally, it can be difficult to differentiate between sarcoidosis and tuberculosis, particularly in those patients who come from areas of high tuberculosis prevalence.

55. What part does stress play in relapse of sarcoidosis occurrence?

Stress can increase the misery caused by the disease.

56. How does sarcoidosis affect the mental attitude of the patient?

Any chronic illness can affect mental health. Individuals become frustrated, tired and depressed. Sarcoidosis can affect the central' nervous system, but severe psychological and psychiatric symptoms are not caused by sarcoidosis.

57. 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, although it may occur along with night sweats, chills and high fevers. But every attempt should be made to exclude infections, particularly tuberculosis, and lymphoma that cause fever.

58. Is the reappearance of nodules or bumps on arms, ears. and legs a sign of sarcoidosis recurrence or flare up?

Reappearance of skin lesions in a sarcoidosis patient indicates flare-up or recurrence.

59. If a person is in remission. how often should he get a lung function study and chest x-ray film?

Once a year.

60. How often should bone density be checked?

If you are on high dose prednisone, check bone density every 6 months.

61. Can joint pain and swelling last for an indefinite period?

Yes, in a small number of the patients, mild joint pains, muscle aches, and stiffness may last for a long time. Deforming joint disease is uncommon in sarcoidosis.

62. Sarcoidosis is found more frequently in African-American patients. As a member of this group what can I do to prevent the progression of the illness?

Since the cause is not known, one can do little to prevent the illness. In African-American patients, the disease tends to be chronic and responds moderately to treatment. It is important for you to find a sarcoidosis specialist who has seen a great deal of sarcoidosis. A combination of various drugs that are commonly used can keep sarcoidosis under control for decades.

63. Can you have sarcoidosis in other organs but the chest x-ray may be normal.

Yes, in ten percent of sarcoidosis patients the 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 high resolution computerized tomography (HRCT) examination of the chest is indicated.

64. 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.

65. What is a "local sarcoid reaction"?

It is the presence of granuloma in one tissue or part of the body without any evidence of sarcoidosis in the rest of the body. Any local injury involving a foreign body can produce a granulomatous reaction e.g., tattoos may develop granulomatous response to various inks and dyes used.