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Respiratory Disorders (Expert Forum)
Effectiveness of Imuran for treating pulmonary fibrosis
Answered by
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Effectiveness of Imuran for treating pulmonary fibrosis
by kayne819, May 19, 2004 12:00AM
My soon to be 78 year-old father is currently in the hospital and has been diagnosed with end stage pulmonary fibrosis as a result of taking amiodarone (cordarone) for 17 years. The doctor suggested putting my father on Imuran to treat the fibrosis. My sister was concerned about the toxicity of Imuran because it is used to treat cancer. Can you give me more information about the drug and it's effectiveness on pulmonary fibrosis?
Doctor's Answer
by National Jewish, May 21, 2004 12:00AM
, May 21, 2004 12:00AM
Idiopathic pulmonary fibrosis (IPF) is one form of interstitial lung disease (ILD). This causes inflammation or swelling that leads to scarring or fibrosis in the interstitium of the lungs. The interstitium is the area between the air sacs and the blood vessels. The most common symptoms of IPF are shortness of breath with exercise and a dry cough. Some people also may have a fever, weight loss, fatigue, muscle and joint pain, and abnormal chest sounds, depending upon the cause.
The ultimate goal of treatment is to clear the swelling before it leads to scarring. Once scarring has occurred it cannot be reversed. In general, the improvement that people with IPF have had with the medicine that is currently available is much lower than we would like. Because of this, new approaches for this disease are aggressively being pursued. However at this time the medicines that are used to treat IPF are
· steroids like prednisone and
· cytotoxics like Imuran® (azathioprine) and Cytoxan® (cyclophosphamide).
Steroids like prednisone are frequently the first medicine used. For a small number of people, steroids will help decrease the swelling before it becomes scar tissue. This will cause a dramatic improvement in symptoms. However, some people have only slight improvement with steroids. It can take 6 to 12 weeks of steroids to see improvement. The response to steroids is related to the amount of swelling present. The goal of treatment is that there will be less swelling or at least no further scarring. This is measured by a follow up chest x-ray, exercise tests, and pulmonary function tests.
Cytotoxics like Imuran® (azathioprine) and Cytoxan® (cyclophosphamide) are used when prednisone does not help or the steroid side effects are severe. After all the goal of treatment is to continue to control the swelling with little or no side effects. The side effects from these medicines are much less when they are used to treat IPF because a much lower dose is used than when they are used to treat cancer. Since these medicines are used to treat cancer, they kill inflammatory cells and keep them from working. This helps to decrease swelling in many different areas of the body, including the lungs. Sometimes these medicines are used alone. In some cases they are used along with other anti-inflammatory medicines such as prednisone. Although Cytoxan® (cyclophosphamide) may help more, the side effects of Imuran® (azathioprine) are more manageable. It can take at least 6 months to see improvement with either of these medicines. To identify side effects early blood tests will be done periodically.
It is a difficult decision to treat with medicine that may cause side effects without helping. A pulmonologist at a university medical center is most likely to have the greatest experience in evaluating people with IPF. This would be the best specialist to determine if the potential benefits of these medicines would outweigh the risk of side effects for your father. Please read our Interstitial Lung Disease MedFact at http://www.nationaljewish.org/medfacts/interstitial.html for more information.
Please check with your father’s doctor to see if he would benefit from using oxygen and participating in pulmonary rehabilitation. IPF can make it difficult for the lungs to get enough oxygen into the blood. When the oxygen level is chronically low the workload on the heart is increased. This causes the heart to enlarge. Once the heart is enlarged it cannot be reversed. Supplemental oxygen is used to prevent the enlargement of the heart. An oximeter is a machine that measures the oxygen saturation of the blood using a sensor that clips to the finger or earlobe. This is a simple test that is used to tell if oxygen is needed. Generally this test is done while sitting and while walking inside the doctor’s office for a few minutes. It can also be done through the night during sleep.
Pulmonary rehabilitation is a program to improve your father’s quality of life through aerobic exercise done on a regular basis. When done routinely, this conditions the entire body so that the muscles are working as efficiently as possible. This allows the breathing muscles to use the least amount of energy and oxygen to move air in and out of the lungs. Over time this helps to lessen shortness of breath. Some programs will also include education about his lung condition and nutrition. Please read our Pulmonary Rehabilitation MedFact at http://www.nationaljewish.org/medfacts/pulmonary.html for more information. Share this information with your father’s doctor to see if this would be helpful and where in his area there are programs.
The ultimate goal of treatment is to clear the swelling before it leads to scarring. Once scarring has occurred it cannot be reversed. In general, the improvement that people with IPF have had with the medicine that is currently available is much lower than we would like. Because of this, new approaches for this disease are aggressively being pursued. However at this time the medicines that are used to treat IPF are
· steroids like prednisone and
· cytotoxics like Imuran® (azathioprine) and Cytoxan® (cyclophosphamide).
Steroids like prednisone are frequently the first medicine used. For a small number of people, steroids will help decrease the swelling before it becomes scar tissue. This will cause a dramatic improvement in symptoms. However, some people have only slight improvement with steroids. It can take 6 to 12 weeks of steroids to see improvement. The response to steroids is related to the amount of swelling present. The goal of treatment is that there will be less swelling or at least no further scarring. This is measured by a follow up chest x-ray, exercise tests, and pulmonary function tests.
Cytotoxics like Imuran® (azathioprine) and Cytoxan® (cyclophosphamide) are used when prednisone does not help or the steroid side effects are severe. After all the goal of treatment is to continue to control the swelling with little or no side effects. The side effects from these medicines are much less when they are used to treat IPF because a much lower dose is used than when they are used to treat cancer. Since these medicines are used to treat cancer, they kill inflammatory cells and keep them from working. This helps to decrease swelling in many different areas of the body, including the lungs. Sometimes these medicines are used alone. In some cases they are used along with other anti-inflammatory medicines such as prednisone. Although Cytoxan® (cyclophosphamide) may help more, the side effects of Imuran® (azathioprine) are more manageable. It can take at least 6 months to see improvement with either of these medicines. To identify side effects early blood tests will be done periodically.
It is a difficult decision to treat with medicine that may cause side effects without helping. A pulmonologist at a university medical center is most likely to have the greatest experience in evaluating people with IPF. This would be the best specialist to determine if the potential benefits of these medicines would outweigh the risk of side effects for your father. Please read our Interstitial Lung Disease MedFact at http://www.nationaljewish.org/medfacts/interstitial.html for more information.
Please check with your father’s doctor to see if he would benefit from using oxygen and participating in pulmonary rehabilitation. IPF can make it difficult for the lungs to get enough oxygen into the blood. When the oxygen level is chronically low the workload on the heart is increased. This causes the heart to enlarge. Once the heart is enlarged it cannot be reversed. Supplemental oxygen is used to prevent the enlargement of the heart. An oximeter is a machine that measures the oxygen saturation of the blood using a sensor that clips to the finger or earlobe. This is a simple test that is used to tell if oxygen is needed. Generally this test is done while sitting and while walking inside the doctor’s office for a few minutes. It can also be done through the night during sleep.
Pulmonary rehabilitation is a program to improve your father’s quality of life through aerobic exercise done on a regular basis. When done routinely, this conditions the entire body so that the muscles are working as efficiently as possible. This allows the breathing muscles to use the least amount of energy and oxygen to move air in and out of the lungs. Over time this helps to lessen shortness of breath. Some programs will also include education about his lung condition and nutrition. Please read our Pulmonary Rehabilitation MedFact at http://www.nationaljewish.org/medfacts/pulmonary.html for more information. Share this information with your father’s doctor to see if this would be helpful and where in his area there are programs.
