What is it?
Fibrosing alveolitis is a condition caused by cells that are normally involved in the body's own defence against infection, instead causing inflammation, injury, and scarring in the lungs. Scars serve a good purpose in the skin where they heal injured areas, but in the lung, scar tissue prevents the lung performing its normal function of taking oxygen from the air into the blood and removing carbon dioxide from the blood.
Who gets it?
Fibrosing alveolitis seems to be on the increase although it is not clear why this is so. It can affect people of any age, but the most common age at which the disease strikes is in the 50s, and men and women are equally affected. The cause of fibrosing alveolitis is unknown, but it is not an infection (and so cannot be caught from others), nor is it a form of cancer.
Exposure to certain occupational dusts (e.g. asbestos, hard metal alloy), can produce disease which is identical to fibrosing alveolitis, and most people with the disease are or have been cigarette smokers. However, for most people the specific cause or provoking factors cannot be identified.
What are the symptoms?
The most common symptom is breathlessness, particularly on exercise such as walking up hills or stairs. Because it may come on in the mid 50s, patients often attribute this to middle age. It is important to stress that the onset of breathlessness should be investigated, rather than to assume it is merely part of an ageing process. If it remains untreated, the condition can often worsen and lead to permanent and progressive breathlessness.
Less common symptoms include a dry cough, and some people may notice a change in the shape of their finger and toe nails. This feature is present in the majority of patients who develop this lung disease.
Occasionally the first indication of fibrosing alveolitis may be abnormalities seen on a chest x-ray performed for an unrelated reason.
What tests will the Doctor want to do?
Because fibrosing alveolitis is one of the less common lung diseases (approximately 5 per 100,000 of the population are believed to suffer from fibrosing alveolitis, although many cases may go undiagnosed) investigations require a referral to a specialist.
Investigations usually include a chest radiograph and a set of breathing tests. These require the individual to breathe in and out of a number of machines which allow an assessment to be made of how well the lungs are working. Blood tests are also usually performed. A special form of x-ray (known as a CT scan) which produces a three dimensional picture of the lungs may be requested.
It may be necessary to obtain samples of lung tissue for examination in the laboratory. This is usually done by bronchoscopy, which involves passing a small flexible telescope down the breathing tubes with the patient lightly sedated. For many people, these tests provide the necessary information for planning treatment, but in some people a larger sample of lung tissue, taken by a surgeon under a general anaesthetic, is needed before treatment can be considered.
What is the treatment?
Fibrosing alveolitis usually requires treatment, even though symptoms appear to be mild. This is because there is already substantial inflammation in the lungs by the time symptoms occur. It is very important that the disease is identified and investigated at the earliest possible stage, so that treatment to reduce progressive lung scarring can be considered at a time before the patient is severely disabled. It is widely recognised that treatment is more effective when it is given before extensive lung scarring has occurred. The type of treatment recommended depends on the results of the tests outlined above.
The most common form of treatment is steroids, usually a short period of high dose therapy followed by a longer period of treatment on lower doses. A few people may experience side-effects. Therefore a very careful balance between the potential benefit of treatment and the risk of side-effects needs to be weighed up before recommendations are made.
Other drugs which may be used to treat the disease are known as immuno-suppressants. This means that these drugs suppress the body's defence mechanisms which are believed to be contributing to the disease, but they can also result in increased susceptibility to infection. People taking these drugs need to have their blood checked on a regular basis, to make sure that they are not receiving too much treatment.
Response to treatment is assessed by changes in symptoms, and in the results of some of the above tests, which may need to be repeated. To achieve this goal, patients are usually seen on a regular basis. Once maximum response has been achieved, the aim is to maintain that response while reducing therapy.
Treatment is usually lifelong. This is because the condition is suppressed rather than cured in the majority of cases, and the long-term aim is to keep the disease suppressed on the smallest possible dosage of treatment. Very occasionally, drugs may be discontinued completely, but this is the exception rather than the rule.
Fibrosing alveolitis is a condition caused by cells that are normally involved in the body's own defence against infection, instead causing inflammation, injury, and scarring in the lungs. Scars serve a good purpose in the skin where they heal injured areas, but in the lung, scar tissue prevents the lung performing its normal function of taking oxygen from the air into the blood and removing carbon dioxide from the blood.
Who gets it?
Fibrosing alveolitis seems to be on the increase although it is not clear why this is so. It can affect people of any age, but the most common age at which the disease strikes is in the 50s, and men and women are equally affected. The cause of fibrosing alveolitis is unknown, but it is not an infection (and so cannot be caught from others), nor is it a form of cancer.
Exposure to certain occupational dusts (e.g. asbestos, hard metal alloy), can produce disease which is identical to fibrosing alveolitis, and most people with the disease are or have been cigarette smokers. However, for most people the specific cause or provoking factors cannot be identified.
What are the symptoms?
The most common symptom is breathlessness, particularly on exercise such as walking up hills or stairs. Because it may come on in the mid 50s, patients often attribute this to middle age. It is important to stress that the onset of breathlessness should be investigated, rather than to assume it is merely part of an ageing process. If it remains untreated, the condition can often worsen and lead to permanent and progressive breathlessness.
Less common symptoms include a dry cough, and some people may notice a change in the shape of their finger and toe nails. This feature is present in the majority of patients who develop this lung disease.
Occasionally the first indication of fibrosing alveolitis may be abnormalities seen on a chest x-ray performed for an unrelated reason.
What tests will the Doctor want to do?
Because fibrosing alveolitis is one of the less common lung diseases (approximately 5 per 100,000 of the population are believed to suffer from fibrosing alveolitis, although many cases may go undiagnosed) investigations require a referral to a specialist.
Investigations usually include a chest radiograph and a set of breathing tests. These require the individual to breathe in and out of a number of machines which allow an assessment to be made of how well the lungs are working. Blood tests are also usually performed. A special form of x-ray (known as a CT scan) which produces a three dimensional picture of the lungs may be requested.
It may be necessary to obtain samples of lung tissue for examination in the laboratory. This is usually done by bronchoscopy, which involves passing a small flexible telescope down the breathing tubes with the patient lightly sedated. For many people, these tests provide the necessary information for planning treatment, but in some people a larger sample of lung tissue, taken by a surgeon under a general anaesthetic, is needed before treatment can be considered.
What is the treatment?
Fibrosing alveolitis usually requires treatment, even though symptoms appear to be mild. This is because there is already substantial inflammation in the lungs by the time symptoms occur. It is very important that the disease is identified and investigated at the earliest possible stage, so that treatment to reduce progressive lung scarring can be considered at a time before the patient is severely disabled. It is widely recognised that treatment is more effective when it is given before extensive lung scarring has occurred. The type of treatment recommended depends on the results of the tests outlined above.
The most common form of treatment is steroids, usually a short period of high dose therapy followed by a longer period of treatment on lower doses. A few people may experience side-effects. Therefore a very careful balance between the potential benefit of treatment and the risk of side-effects needs to be weighed up before recommendations are made.
Other drugs which may be used to treat the disease are known as immuno-suppressants. This means that these drugs suppress the body's defence mechanisms which are believed to be contributing to the disease, but they can also result in increased susceptibility to infection. People taking these drugs need to have their blood checked on a regular basis, to make sure that they are not receiving too much treatment.
Response to treatment is assessed by changes in symptoms, and in the results of some of the above tests, which may need to be repeated. To achieve this goal, patients are usually seen on a regular basis. Once maximum response has been achieved, the aim is to maintain that response while reducing therapy.
Treatment is usually lifelong. This is because the condition is suppressed rather than cured in the majority of cases, and the long-term aim is to keep the disease suppressed on the smallest possible dosage of treatment. Very occasionally, drugs may be discontinued completely, but this is the exception rather than the rule.
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