This section contains snippets of information about sarcoidosis and links to sites where you may read more detailed accounts of the disease. The information I have provided here was selected from topics in which I was interested when first diagnosed and topics of searches done by visitors to my site since I started it. If anyone has a subject he/she would like to have me add to this section, please feel free to contact me.

SPECIAL NOTE: Dr. Om P. Sharma, a pulmonary specialist and Professor of Medicine, Keck School of Medicine at USC-LAC, Los Angeles CA, has assembled an informative brochure of FAQ’s about sarcoidosis. Until recently he sent the brochure to individuals via the postal service, but he has recently assembled it on his Website. For further substantial information about sarcoidosis, you may view the brochure, "Sarcoidosis: Commonly Asked Questions", on Dr. Sharma’s Website, "SarcoidosisSharma.com", or download it to your own computer to read at your leisure.

• Sarcoidosis is a chronic, systemic disease. Of those who contract it, a large majority, believed to be approximately 80%, will never experience further episodes or exacerbations after the initial disease period for which they are diagnosed. Within a year or two they will be completely free of symptoms. They will, however, never be completely cured. The disease has no known cure and will remain in the individuals' bodies for the rest of their lives. The remainder of those who contract sarcoidosis will continue to have symptoms and/or flare-ups of disease activity in varying levels of severity. Some will have minor symptoms or possibly no visible symptoms, but will have abnormal readings on medical tests done to indicate disease activity. Others may have erratic periods of active involvement of the disease in one or more of their bodily parts/organs, followed by a period of inactivity in which they may have no symptoms. And others yet will continue to have some symptoms despite the fact that the disease has become inactive. A small proportion of those diagnosed with sarcoidosis will grow progressively sicker and require considerable medical intervention to maintain their health to the highest possible degree. Despite all medical assistance, about 5% of those diagnosed with sarcoidosis will succumb to its debilitating effects and die.

  
  

• Many physicians believe that sarcoidosis is a benign condition, but the doctors at the National Jewish Medical and Research Center do not believe that is always true. They feel that doctors should remain vigilant in diagnosing and managing sarcoidosis. A small number of patients will have a very gradual, insidious progression of the disease, which results in permanent damage to one or more areas of the body. Other patients will have an inactive level of sarcoidosis, but will continue to exhibit some ongoing symptoms during that period. Therefore, once a patient is diagnosed with sarcoidosis, he/she should be evaluated by a doctor regularly whether symptoms are present or not. Appropriate tests for the most reliable indicators of sarcoid activity, including a complete eye exam, should be included in such evaluations. If stability of the condition and signs of inactivity have been maintained for longer than one year after the cessation of treatment, the possibility of relapse is considerably diminished, but the patient and his physician should continue to be vigilant, since relapses can occur even after ten years of inactivity.

  
  

• The actual origin of sarcoidosis is unknown, but it is believed that it may involve a combination of a dominant genetic trait that is very common (20% of the population) and an agent in the environment such as an allergen or virus that triggers the process. The National Jewish Medical and Research Center states that it appears that genetically predisposed hosts are exposed to antigens that trigger an exaggerated cellular response and the formation of granulomas.

  Sarcoidosis is an autoimmune disease, and, according to the American Autoimmunce Related Diseases Association autoimmune diseases may occur in families, but may present as one of about a hundred different autoimmune diseases (in my case, my daughter was diagnosed with mulitple sclerosis about the same time my initial symptoms appeared). Therefore, it is important for families with members who have autoimmune diseases to mention this fact when another member of the family is experiencing medical problems that appear to be difficult to diagnose.

  
  

• The Illinois Department of Public Health reports that "although sarcoidosis was once considered a rare disease, it is now known to be a chronic, common illness that appears all over the world. In fact, it is the most common of the fibriotic lung disorders." There are two possible reasons for the belief that it is rare. First, it seems that many patients have no visible symptoms and appear to be healthy. They are diagnosed "by accident" while being assessed for some other medical problem or having a routine physical exam. Secondly, some patients are misdiagnosed as having any of a number of diseases with similar symptoms. Additionally, the medical community and general public do not seem to have a great deal of awareness about the disease. They need to be provided with information, so that they will become better informed about sarcoidosis. This can only be beneficial to those who have contracted this disorder and should encourage the provision of greater funding for research about the disease, its origins, treatment, and possible cures.

  
  

• A number of general symptoms may appear in those who have sarcoidosis. These include fatigue that may be extreme at times and require the patient to get additional amounts of sleep, including naps during the day. There may be varying degrees of depression that sometimes have a sudden onset/retreat. Muscle weakness/lethargy may be present as well as general joint pain, most often in the knees. Low-grade fever, heat sensitivity, weight loss, and headaches are additional symptoms that may occur in sarcoidosis. In a review of a study of sarcoid patients, Ruth Wirnsberger, University of Maastricht in the Netherlands, wrote in "Quality of Life and Health Status in Sarcoidosis" that along with the chest-related symptoms, "patients may also suffer from nonspecific symptoms such as fatigue, fever, weight loss, and muscle pain." "Persistent fatigue may not be taken seriously or may be underestimated by the patient's physician, family, and/or workplace." "Moreover, about one third of the patients suffered from tension, strain, and/or depressive symptoms, which by the patients themselves were attributed to the ongoing disorder." Ms. Wirnsberger stated that patients with sarcoid were found to be impaired with respect to their mobility, sleep, ability to work, recreation/exercise, and social interaction. Where fatigue was a factor, metabolic derangements and decreased respiratory muscle strength were found.

  Site specific symptoms depend upon the body system or organ involved. The most frequently involved organs are the lungs. An abnormal chest x-ray may or may not be accompanied by such overt symptoms as shortness of breath (especially upon exertion), heaviness/tightness in the chest, and a dry, non-productive cough. These often result in a reduced exercise capacity. When the nervous system is involved there may be a myriad of symptoms possible (see discussion below). Heart involvement is often silent and may only be detected after death. Ocular involvement is quite frequent, so anyone suspected of having sarcoidosis should have a complete eye examination at least once a year in order to prevent any unnecessary loss of sight. Eye involvement may not be heralded by any overt symptoms at onset, but may also be indicated by somewhat dry, scratchy eyes. The most common eye condition is uveitis. Skin conditions may vary as to type of lesion, body location, and severity/longevity of the symptoms.

  
  

• Sarcoidosis quite frequently appears in the lungs initially, and about 90% of all individuals who are diagnosed with Sarcoidosis eventually will have lung involvement. About half of these patients with more acute presentations will have a combination of symptoms that together are called Lofgren's Syndrome. They include bilateral hilar (lymph)adenopathy, which is an inflammation/enlargement of the lymph nodes in the lungs, and erythema nodosum, which includes painful red, raised nodules that appear on the skin, most often on the front of the leg between the knee and ankle (frequently found in women in the springtime). These symptoms may be accompanied by polyarthralgias (multiple joint aches), fatigue/general weakness, and low-grade fever. The joint pain does not usually result in arthritis that can cause destructive joint changes. The predicted outcome of this condition is favorable, and most patients will be entirely symptom-free within two years and experience no further problems.

  
  

• Pulmonary (respiratory) sarcoidosis is classified in five stages (categories) based on chest x-ray findings (CXR). It is important to emphasize that the term, stages, can be misleading in that patients do not always graduate from a mild case to a more serious case. Stage one does not necessarily progress to stage two, three etc. This is why some physicians refer to these categories as types or groups rather than stages, since they do not imply a progression; rather they are patterns seen on the x-rays. These categories have been useful for prognosis (likelihood of improvement), but have a low correlation with symptoms and pulmonary function. In many cases the disease remits and never returns.

  Stage 0 -- Normal chest radiography (Chest X-ray or CXR)
  

  Stage 1 -- Bilateral hilar lymphadenopathy (disease condition of the hilar lymph nodes usually occurring on both sides simultaneously)
  

  Stage 2 -- Bilateral hilar lymphadenopathy (see Stage 1 for explanation) with nonfibrotic pulmonary parenchymal disease (See Stage 3 for explanation)
  

  Stage 3 -- Nonfibrotic pulmonary parenchymal disease (disease of the essential lung elements but without formation of fibrous or scar tissue)
  

  Stage 4-- Fibrosis with distortion of lung and cavitary changes (formation of fibrous tissue that replaces the essential lung elements and causes changes and distortions in lung/lung cavity)

  A lengthy and in-depth article, "Sarcoidosis, A Primary Care Review" includes a table that provides information about the differences in chest radiographs, pulmonary function tests, signs/symptoms, and expectation of remission for the various stages of pulmonary sarcoidosis. The article appears in the December 1998 issue of The American Family Physician magazine.

  
  

• Approximately 10% of patients will have neurologic involvement according to Dr. DeRemee of the Mayo Clinic. Nodules can infiltrate the nervous system, causing many symptoms including loss of/abnormal sensations, loss of muscle strength, and joint stiffness. Brain or cranial nerve (central nervous system) involvement includes such symptoms as facial palsy, mental disturbances (confusion, disorientation, loss of memory/judgment/ability to calculate), seizures, headaches, changes in various sensory abilities (taste, smell, hearing, vision), and speech impairment. It may involve the development of space-occupying masses (lesions) that will cause diverse neurologic abnormalities depending on their location. In order to relieve the symptoms and improve quality of life for the patient with such abnormalities, the mass will have to be removed. If the pituitary gland becomes affected, there may be a high urine output, excessive thirst, excessive fatigue, and changes in menstrual periods for women. Any part of the nervous system may become affected by neurosarcoidosis. Such involvement may be a single nerve, multiple nerves, or generalized. If unusual combinations of neurologic deficits affect the central nervous system or peripheral nerves (or both), then a clinical suspicion of Sarcoidosis should be raised. If you have Sarcoidosis and believe you are having neurological symptoms, you should contact your physician.

  
  

• There is no single medical test or procedure that will absolutely diagnose the presence of sarcoidosis in a person. Normally, various tests and procedures are conducted, both to rule out other disease conditions and to confirm the presence of sarcoidosis in the patient. In addition, a complete medical history taking and thorough physical examination are a vital part of the diagnosis process.

  Some of the best known tests and procedures that may be performed include: a chest x-ray, CT scan, pulmonary function tests, bronchoscopy/transbronchial biopsy, and a gallium scan, all of which involve the lungs. Other tests that should/could be performed include: ophthalmic evaluation with slit lamp exam (eyes), electrocardiogram and thallium scan (heart), tuberculin and Kveim-Siltzbach tests (skin), and a biochemical panel (urine and blood) to evaluate kidney/liver functions and measure calcium levels.
  
  

  Blood and urine tests are usually among the first steps taken to determine what, if any, medical problems a person may be experiencing. The complete blood count (CBC) will likely be done as well as a test to determine the level of serum angiotensin converting enzyme serum (SACE or ACE) in the blood, as that is an indicator of active sarcoidosis in many individuals. Along with the basic non-sterile urine sample you may provide before leaving the doctor's office, you may also be asked to collect your urine for a 24-hour period. This collection will be used to determine the level of calcium in your urine. Formation of excessive calcium in the bodies of those with sarcoidosis does occur in some instances and needs to be treated before severe complications arise.

  The chest x-ray (CXR) is often one of the first procedures performed along with general blood and urine tests. It will provide the first general impression as to whether there are any abnormalities present in the lungs. When sarcoidosis is suspected, a Gallium scan may also be performed. The patient receives an intravenous injection of radioactive gallium and then returns 1-2 days later to be "scanned" by a machine that moves up and down above the body as the patient lies on an examination table.

  A CT scan (computed tomography), also called a CAT scan, of the thoracic (chest) area is performed by having the patient lie on a special platform that is rolled into a large "tube" where the "pictures" are taken. The patient's head will be near the opening of the machine. The only discomfort is from the hardness of the platform, the loudness of the machine, and the need to be very still and hold one's breath for certain intervals.

  Pulmonary function tests (PFTs) are breathing tests in which the patient must breath into various devices (by mouth) in various ways to measure breathing ability and lung capacity. Since 90% of all sarcoidians have lung involvement at some time and the level of involvement or activity may not be indicated on a lung x-ray, the PFTs can often assist in determining how much breathing impairment, if any, has occurred/is occurring.

  A tuberculin skin test may be done to rule out the likelihood of tuberculosis. An additional skin test to determine the presence of sarcoidosis is sometimes done, but with decreasing regularity. It is called a Kveim-Siltzbach (or just Kveim) skin test. Sarcoid tissue, often from the spleen of someone who has sarcoidosis, is injected under the skin, and the results are read several weeks later to determine whether an granulomatous tissue has formed at the site.

  Biopsy of lung tissue samples is often the most conclusive way to diagnosis sarcoidosis. The initial biopsy is usually a transbronchial biopsy that is performed in conjunction with a bronchoscopy. The patient will be lightly sedated and given some medication to numb the throat area. Then a tube with a small, lighted scope is inserted, usually through one of the nasal passages, and threaded into the lungs. Small tissue samples are taken during this procedure, but if they do not confirm/deny the presence of sarcoidosis, an open lung biopsy (via mediastinoscopy) may be required to verify diagnosis. During this procedure, the patient is anesthetized and an incision is made near the top of the breastbone. Larger tissue samples may be obtained from the lungs through the incision than via the bronchoscopic tube.

  In all cases of suspected or confirmed sarcoidosis a complete eye examination is recommended. Sarcoidosis can often affect the eyes in a variety of ways--the most common condition is uveitis--that may not be readily detectable by the patient until permanent damage has occurred. Therefore, it is important that you have your eyes checked on a regular basis if you have sarcoid. Be sure to ask that the doctor perform a slit lamp exam during the visit.

  Since cardiac involvement in sarcoidosis is most often determined only after a person dies from its effects, some doctors may wish to perform cardiac tests such as an electrocardiogram, thallium heart scan, etc. if there is any sign that the heart is involved. Other tests may be ordered based on symptoms relating to specific parts of the body that are suspected as having sarcoid involvement.

  
  

• Corticosteroids are the most common medications used to treat Sarcoidosis. They appear to be the most effective in relieving many of the symptoms of the disease. The most common corticosteroid is Prednisone, which can cause significant side effects and should only be used under the strict advice and supervision of your physician. Sometimes oral Prednisone is prescribed to be taken on alternating days so that the side effects are minimized. Inhaled corticosteroids, which are generally prescribed for chronic lung problems, are sometimes recommended over oral corticosteroids because they remain locally in the airway and thus cause fewer and lesser side effects (rinse and gargle with water after each use to prevent thrush; usage may also cause hoarseness and/or sore throat). The drawback is that there is little evidence that use of these inhaled corticosteroids results in any long-term improvement of sarcoid symptoms. Two of these inhaled corticosteroids include budesonide, which is known as Pulmicort in Australia and Rhinocort in the United States, and fluticasone propionate, which is known as Flixotide in Australia and Flovent or Flonase in the United States. Names of other corticosteroids include: triamcinolone, prednisolone, dexamethasone, beclomethasone, and deflazacort.

  According to an article in Chest that was published in the March 1997 issue, authors Gottlieb, Israel, Steiner, Triolo, and Patrick stated that "74% of patients who were adequately treated with systemic corticosteroids for severe symptoms of sarcoidosis appeared to be at lifetime risk for recurrence of disease. Because of the risk of relapse, we recommend at least semiannual visits for those patients who are or who have been treated with systemic corticosteroids. If corticosteroids predispose to clinical relapse, the implication would be to withhold treatment from patients with newly diagnosed sarcoidosis unless symptoms and disability were compelling, and a similarly conservative approach would be appropriate during relapse."

  Important Note: The Arthritis Foundation (among others) advises that people who take corticosteroids should NEVER decrease or stop them abruptly. Ceasing use of the drug must occur slowly with close supervision by a doctor. The reason is that the body does not produce its own cortisone when corticosteroid medications are present. If the drug was stopped abruptly, there would be no cortisone present in the body. This could be fatal. When corticosteroids are stopped gradually, over a period of weeks to months, it gives the body time to adjust and start producing cortisone on its own again. Patients taking corticosteroids should carry a card or preferably wear a bracelet indicating that they are taking a corticosteroid.

  Other medications that may provide relief from some of the symptoms of sarcoidosis, but do not appear to have the overall beneficial effects of corticosteroids, include methotrexate, azathioprine, cyclophosphamide, and cyclosporine, which are immunosuppressives. Additional medications used include chlorambucil, hydroxychloroquine, chloroquine, colchicine, oxyphenbutazone, P-aminobenzoic acid, sulphathiazole, calcitonin, dutinulan 8-15, allopurinol, indomethacin, levamisole, and D-penicillamine. The full impact of most of these medications is still being reviewed, so be sure to carefully discuss with your doctor any that you may consider using.

  
  

• Uveitis, is one of the more common conditions related to having sarcoidosis and occurs in about 25% of those who have the disease. According to information provided on the WellnessWeb, it is an inflammation of the uvea, the pigmented layer of the eye. The uveal tract consists of the iris (the colored part of the eye), the ciliary body (behind the iris and responsible for manufacturing the fluid inside the eye), and the choroid (the vascular lining tissue underneath the retina). Inflammation of the nearby retina (retinitis) is considered to be in the category of uveitis.

  Dr. C. Stephen Foster, Director of the Department of Uveitis/Ocular Immunology at the Massachusetts Eye & Ear Infirmary, states that uveitis is the third leading cause of blindness in the United States. He goes on to say that approximately 60 different things can cause uveitis. Both infectious and non-infectious as well as malignant causes exist, but uveitis based on autoimmunity is the most common form and tends to be recurrent. Uveitis is the most common eye problem encountered in people who have sarcoidosis.

  The Casey Eye Institute, Department of Ophthalmology, which is located on the campus of the Oregon Health Sciences University, contains the Uveitis Clinic. Its Website contains substantial information about treatments for uveitis and contains a very nice diagram of the eye and its parts. The Casey Eye Institute is one of the world's leading uveitis research centers and explains uveitis as follows. "Inflammation can occur anywhere in the body. In general, inflammation is the body's response to an injury. Inflammation always involves the recruitment of white blood cells from circulation to the site of injury. White blood cells normally circulate in the body and are vigilantly looking for an injury such as an infection or a wound. Although, uveitis can result from an infection, for most patients, uveitis is thought to be occurring because white blood cells are responding to a signal that injury is occurring even though no detectable infection is present. Since white blood cells are the major component of one's immune system, often times uveitis is appropriately described as an autoimmune disease, a disease in which the body's own immune system attacks or betrays itself."

  An article about Uveitis at the HealthCentral Web site explained that the uvea is the middle layer of the eye, is very vascular and supplies blood to the retina. The most common form of uveitis is anterior uveitis which affects the iris at the front of the eye. This condition is also referred to as iritis or nongranulomatous uveitis. The disorder is associated with some autoimmune diseases, may affect only one eye, and is most common in young or middle-aged people.

  Posterior uveitis affects the back of the uveal tract and may involve the choroid cell layer or retinal cell layer or both. This condition is also known as granulomatous uveitis, choroiditis, choroidoretinitis, or sometimes just retinitis. The inflammation causes spotty areas of scarring that correspond to areas with vision loss. The degree of vision loss depends upon the amount and location of the scarring. It can involve one or both eyes and is the form of uveitis most often associated with sarcoidosis and a number of other specific diseases. Posterior uveitis is likely to last for months or even years and may cause permanent vision damage even with early treatment.

  Important Note: If a person is even suspected of having sarcoidosis, he/she should have a complete eye examination including a slit lamp exam. If sarcoidosis is confirmed, the individual should have a minimum of yearly eye exams and should have an exam at any other time that there is unusual dryness, scratchiness, or other symptoms that are out of the ordinary. Uveitis can often be quite advanced by the time obvious symptoms appear. Therefore, it is very important for individuals to act promptly if they have sarcoid and notice any unusual sensations in their eyes.