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SARCOIDOSIS

When was sarcoidosis discovered?
There has been an awareness of sarcoidosis for more than 100 years since it was first described by a London surgeon-dermatologist, Dr. Jonathan Hutchinson in 1877. The doctor described the findings of a 50 year-old man who had large purple skin plaques on the hands and feet and a 64-year-old woman with large purple patches on her face and arms.
In 1889, a Norwegian dermatologist Dr. Cesar Boeck named the process "multiple benign sarcoid of the skin". He also showed that many patients also had sarcoid in the lymph nodes and lungs.
In 1958, scientists from all over the world met at the Brompton Hospital in London for a conference about sarcoidosis. Since then there has been a yearly international sarcoidosis conference. There is a scientific journal for sarcoidosis. The search for the cause and the search for new treatments continue at an intense pace.

Who gets sarcoidosis?
Anyone, anywhere in the world can develop sarcoidosis. There may be variation from country to country, but in general it occurs in men and women equally. It can occur in individuals who smoke or do not smoke. Sarcoidosis often occurs during the ages of 20 to 40, and 70% of patients are under 40 years of age, but it may occur at all ages. The disorder can occur at any time of the year.

What causes sarcoidosis?
The cause of sarcoidosis is not known; however, this diagnosis is what is known as a "diagnosis of exclusion". This term is used for a disease that has no known cause, but mimics other disorders that must first be excluded before establishing the diagnosis of sarcoidosis.
For example, there is a lung disease called hypersensitivity pneumonia (HP), which is also referred to as allergic alveolitis that must be excluded. This is an acute or chronic granulomatous lung disorder that is caused by an immune response to a variety of organic substances (antigens) that include bird proteins, spores of microbes, or airborne bacteria. There are over 300 agents that have been reported to cause this disorder. Hypersensitivity pneumonia may occur as a reaction to protein material from pigeons (pigeon breeder's disease) or parakeets, from contaminated humidifiers or air conditions in the home, office or automobile (humidifier lung), or from mists created from contaminated water such as a basement shower or sauna.
Hypersensitivity pneumonia may have the same symptoms and identical chest x-ray findings as sarcoidosis. A blood serology test that is positive for the causative agent can diagnose hypersensitivity pneumonia. Avoidance of the causative agent can result in resolution of the illness. Sometimes, a brief course of corticosteroid treatment is utilized in the acute form of hypersensitivity and a longer course for the chronic form.
Chronic fungal infections of the lung such as histoplasmosis can also mimic sarcoid in certain situations, and these disorders must also be excluded. The fungal disorders are treated with a long course of specific types of antibiotics.
Exposures to certain metal dusts can cause a disorder indistinguishable from sarcoidosis. In the late 1940's there was a group of patients with "sarcoid" in the region of Salem, Massachusetts. It was even called "Salem Sarcoid". Dr. Harriet Hardy investigated the disorder and discovered that it occurred only in employees working in a factory where the metal, beryllium, was used to increase the duration of use of fluorescent light bulbs. This disorder was called berylliosis. The use of beryllium in fluorescent lights was stopped after the description of the disorder and no more cases of "Salem Sarcoid" occurred. Avoiding exposure to beryllium can result in resolution of the acute form of the illness, although, a course of corticosteroid treatment is generally utilized. The chronic form is more difficult to treat.

Does sarcoidosis occur outside of the lung?
Sarcoidosis occurs in the lung or the lung-related lymph nodes in 90% of patients. In some of these patients, there may also be sarcoidosis in one or more other locations such as the lymph nodes, skin, liver, heart, kidneys or the nervous system. Sarcoidosis without involvement of the lung occurs in less than 10% of patients.
Specifically, sarcoidosis occurs in the lymph nodes in the hilar regions of the lungs in 75% to 90% of patients and in the lungs themselves in 50% of patients. Sarcoidosis involves the lymph nodes elsewhere in the body in 30% of patients, the eyes in 20%, the skin in 20%, the heart in 10%, the bones in 10% and the neurological system in less than 5 percent of patients.
In 10% of patients, the calcium level in the blood may be increased. In rare situations, the level of calcium may be so high that treatment will be required to decrease the level before an adverse reaction develops.
How do you get sarcoidosis?
No one knows. Sarcoidosis is not contagious. In rare situations, it has been reported to occur in twins and family members, but a consistent inheritance pattern has not been established.
One theory of the mechanism of disease is that an antigen exposure causes dysfunction of the lung immune response resulting in a T-lymphocyte immunity that promotes inflammation and granulomas in the lung.

Is sarcoidosis fatal, can individuals die from it?
The outcome of sarcoidosis depends upon the severity of the illness. It is not common, but in a certain percentage of situations, sarcoidosis can be fatal.
What is the outcome of sarcoidosis of the lung, can it be cured?
The chest x-ray pattern has been classification into three stages of sarcoidosis of the lung. These are not necessarily sequential stages that all patients follow, but they are used to some extent for description of disease activity.
Stage I is limited to lymph node enlargement in the hilar regions. The lungs appear normal.
Stage II shows enlarged lymph nodes in the hilar regions and small nodular opacities in the lungs.
Stage III shows no enlarged lymph nodes but shows linear shadows suggestive of scarring in the upper lungs and sometimes cystic structures. This last stage probably represents a late stage and may represent an inactive process.
Stages I and II and the most common. Stage III is less common.
Outcome varies among these three x-ray patterns. For stage I, 60% to 80% of patients are cured. For stage II the percentage decreases somewhat to 50% to 60%. For stage III, it is the lowest with 30%.
Findings that are associated with a poorer prognosis include onset over age 40 years, symptoms for more than six months, involvement of three or more organs, and stage III pulmonary disease.
Computerized chest x-rays that show a fluffy or "ground glass" appearance and nodular opacities suggest a responsive lesion. Cystic air spaces and architectural distortion of the lung tissues represent irreversible findings.
Serial pulmonary function and chest radiographs are the most helpful tests for monitoring the activity of sarcoidosis of the lung. Other specialized tests are utilized for monitoring sarcoidosis of the heart, eye, kidney or other organs.
The overall outcome for sarcoidosis is fairly good, 50% of patients with sarcoidosis will eventually have complete resolution without residual effects. Among the remaining 50%, there may be permanent organ dysfunction, but it is usually of a mild degree and only detectable by detailed testing.

How is sarcoidosis of the lung treated?
There has been a change in the approach to the management of sarcoidosis for a large group of patients. In the past, patients with the diagnosis of sarcoidosis were treated. Now, patients are categorized into two groups. One group that is treated, and another group that treatment is delayed or not given.
The current emphasis is for a period of observation for patients who do not require treatment for symptoms. Studies have shown that 40% of patients will have spontaneous improvement, 40% of patients will respond to subsequent treatment, and 20% of patients will require immediate treatment.
Corticosteroid therapy remains the standard treatment usually in the form of prednisone. Immediate therapy is usually indicated for patients with stage II and stage III lung disease who have symptoms and pulmonary function test abnormalities. Immediate therapy is also recommended for patients with severe sarcoidosis of the heart, eye, neurological system, or if there is a severely increased calcium level in the blood.
A period of observation, three months or six months, is appropriate for individuals without symptoms, without pulmonary function test abnormalities, and with milder forms of the illness. If no symptoms develop and no new disease activity is detected, continued observation is recommended. A total of two years of observation and monitoring is usually sufficient to establish resolution or an inactive state. If symptoms develop before the scheduled monitoring tests or if symptoms and test findings change at the three-month or six-month interval to a severe category, corticosteroid treatment is begun.
Current treatment protocols indicate the use of 30 to 40 mg of prednisone daily for 8 to 12 weeks, with gradual decreasing of the dose to 10 to 20 mg every other day over a period of 6 to 12 months.
In special situations where sarcoidosis involves the airways and the patient is being treated with prednisone, inhaled corticosteroid therapy may be utilized as a means of decreasing the dose of the prednisone.

Does corticosteroid treatment have side effects?
Yes, the adverse effects can be numerous, most are reversible but some are not. This medication will save a person's life, but can also cause difficulties in some individuals.
The common adverse reactions include increased appetite, weight gain, and bruising of the skin. A rounded puffy face, acne-like skin lesions, "fat pads" below the neck in the back and in the front may develop over time.
Some psychological effects, high blood pressure, diabetes, and osteoporosis (softening of the bones) may develop. Cataracts can occur. A very rare condition known as aseptic necrosis of the hips requiring hip replacement may develop.
It is important to obtain the list of effects and review them.
Some individuals have no difficulty with the medication. Others may be bothered by some of the adverse reactions but can tolerate them or they disappear. Every once in awhile, a person cannot take the medication or develops a severe reaction.
The prednisone is given at the lowest dose that is effective for the shortness length of time as possible. The every other day dosage can decrease the side effects

What if corticosteroid therapy is not effective?
For sarcoidosis, corticosteroid treatment has appeared to improve the radiographic findings, yet whether the medication has an ultimate effect on whether a person is going to have residual scarring has not been proven. For this reason, there has been a change in the approach to treatment as noted above. In some situations, the scarring process may progress.
In these situations if the scarring is combined with a decreased oxygen value, supplemental oxygen can be beneficial. The heart has to work harder because of the increased pressure in the lungs from the fibrosis and soon fails. Oxygen has a direct benefit for the heart. Oxygen will prevent the heart from enlarging and weakening. The heart will not fail. In order to be effective, supplemental oxygen is required for at least 18 hours per day. Generally, it is recommended that the oxygen is utilized 24 hours daily. It seems excessive at first, but individuals become use to it and are able to lead an active and zestful life.
A pulmonary rehabilitation program should also supplement the oxygen program. Exercise is very useful in improved conditioning and efficiency of the muscles from the remaining good lung. The rehabilitation program is useful because an individual can be pushed to a higher level of exertion in a protected environment. The exercise program (e.g., 30 to 45 minutes of daily walking) can be continued on a home basis after the controlled, supervised program.
For patients who are not able to take prednisone or who require high doses of prednisone, methotrexate at 10 mg per week may enable patients to decrease their prednisone use after six months. Methotrexate treatment in some patients may be related to abnormal blood tests and certain infections. It is important to review and discuss possible adverse reactions with the doctor so a person can be aware of a potential reaction early.
There have been anecdotal reports of other treatments. Some of these may appear to be effective on an individual basis, yet studies of a large number of patients are needed to confirm the success of these treatments.
In life threatening, disabling disease and progressive disease that has not responded to treatment, lung transplantation may be utilized