Disease: Pulmonary Fibrosis

    What is pulmonary fibrosis?

    "Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes (sarcoidosis, Wegener's granulomatosis), infections, environmental agents (asbestos, silica, exposure to certain gases), exposure to ionizing radiation (such as radiation therapy to treat tumors of the chest), chronic conditions (lupus, rheumatoid arthritis), and certain medications.

    In a condition known as hypersensitivity pneumonitis, fibrosis of the lung can develop following a heightened immune reaction to inhaled organic dusts or occupational chemicals. This condition most often results from inhaling dust contaminated with bacterial, fungal, or animal products.

    In some people, chronic pulmonary inflammation and fibrosis develop without an identifiable cause. Most of these people have a condition called idiopathic pulmonary fibrosis (IPF) that does not respond to medical therapy, while some of the other types of fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may respond to immune suppressive therapy.

    Synonyms (other names) for various types of pulmonary fibrosis that have been used in the past include chronic interstitial pneumonitis, Hamman-Rich syndrome, and diffuse fibrosing alveolitis.

    What are causes and symptoms of pulmonary fibrosis?

    Symptoms of pulmonary fibrosis include:

    • shortness of breath,
    • coughing, and
    • diminished exercise tolerance.

    Symptoms vary depending on the cause of the pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.

    The most common form, idiopathic pulmonary fibrosis, has slow and relentless progression. Early on, patients often complain of a dry, unexplained cough. Often, slow and insidious onset of shortness of breath can set in. With time, dyspnea (shortness of breath) worsens. Dyspnea initially occurs only with activity and is often attributed to aging. Over time, the dyspnea occurs with little or no activity. Eventually, the shortness of breath becomes disabling, limiting all activity and even occurring while sitting still. In rarer cases, the fibrosis can be rapidly progressive, with dyspnea and disability occurring in weeks to months from the onset of the disease. This form of pulmonary fibrosis has been referred to as Hamman-Rich syndrome.

    How is pulmonary fibrosis diagnosed?

    Pulmonary fibrosis is suggested by a history of progressive (worsening over time) shortness of breath with exertion. Sometimes, during examination of the lungs with a stethoscope, the doctor can hear crackling sounds in the chest. These crackles have a very characteristic sound and are very similar to the sound heard when Velcro is pulled apart. These are often referred to as "Velcro crackles (or rales)". The chest X-ray may or may not be abnormal. However, a special X-ray test called a high resolution CT scan will frequently demonstrate abnormalities. This type of X-ray provides a cross-sectional picture of the lungs in very detailed resolution. The classic findings in idiopathic pulmonary fibrosis show diffuse peripheral scarring of the lungs with small bubbles (known as bullae) adjacent to the outer lining of the surface of the lung, often at the bases of the lungs.

    Lung function testing is distinctly abnormal. The volumes of the lungs may be reduced, as may the airflow, but the characteristic finding is a reduction in the diffusing capacity. The diffusing capacity is a measure of the ability of the lungs to exchange gases (oxygen and carbon dioxide) into and out of the blood stream.

    The diagnosis can be confirmed by lung biopsy. An open surgical biopsy, meaning that the chest wall must be surgically opened under general anesthesia to remove a portion of lung tissue, may be necessary to obtain enough tissue to make an accurate diagnosis. The most common type of biopsy in this situation is by a video assisted thoracoscope. Basically, this involves placing a small tube into the chest cavity through which biopsy samples can be obtained. Often, if the clinical situation is very classical in presentation, a biopsy may be unnecessary. The biopsy specimen is examined microscopically by a pathologist to confirm the presence of fibrosis.

    How is pulmonary fibrosis treated?

    The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition, since scarring is permanent once it has developed. Lung transplantation is the only therapeutic option available. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. Research trials using different drugs that may reduce fibrous scarring are ongoing.

    Since some types of lung fibrosis can respond to corticosteroids (such as prednisone)or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to decrease the processes that lead to fibrosis. These drugs do not help idiopathic pulmonary fibrosis. However, other causes of lung fibrosis may be responsive to immune suppression.

    The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent. Conditions that improve with immune suppressive treatment probably are not idiopathic pulmonary fibrosis.

    The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need and duration of treatment, and will monitor the response to therapy along with any side effects.

    Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids. These include

    • cyclophosphamide (Cytoxan),
    • azathioprine (Imuran, Azasan),
    • methotrexate (Rheumatrex, Trexall),
    • penicillamine (Cuprimine, Depen), and
    • cyclosporine.

    Learn more about: Cytoxan | Imuran | Azasan | Rheumatrex | Trexall | Cuprimine

    The anti-inflammatory medication colchicine has also been used with limited success. Other trials using drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone have not met with much success in the treatment of idiopathic pulmonary fibrosis.

    Learn more about: Cellcept

    Pulmonary fibrosis causes decreased oxygen levels in the blood. A decrease in blood oxygen level (hypoxia) can lead to elevated pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to failure of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are frequently treated with supplemental oxygen to prevent pulmonary hypertension. In some cases, new agents used to lower the blood pressure in the pulmonary artery have had limited benefit.

    There is also evidence that patients suffering from pulmonary fibrosis may be at increased risk for blood clots that travel to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be indicated.

    What are the complications of pulmonary fibrosis?

    Idiopathic pulmonary fibrosis tends to be relentless in its progression. The complications that occur are a reflection of the failure of the pulmonary system. Dyspnea, decreased activity, and signs of heart failure can occur. As the lungs fail, the blood pressure in the lungs rises. This results in increased work for and ultimately failure of the right side of the heart which pumps the blood through the lungs. This failure can result in fatigue, leg swelling, and overall fluid accumulation in the body. The immobility and sluggish blood flow can increase the risks for blood clots. Depression is frequently seen in this devastating disease.

    Can pulmonary fibrosis be prevented?

    Idiopathic pulmonary fibrosis has an increased frequency in cigarette smokers. This is just one more reason not to smoke. The cause of idiopathic pulmonary fibrosis is unknown, and therefore prevention is difficult. There is a rare form of idiopathic pulmonary fibrosis that runs in families. Work is being done at National Jewish Hospital in Denver, Colorado trying to identify markers for this disease.

    Unfortunately, since this is a fatal disease without effective therapy, there are many charlatans trying to take advantage of these stricken individuals and their families. There is no evidence that special diets or supplements or bowel preparations will help this disease in any way.

    What is the prognosis for pulmonary fibrosis?

    The prognosis of this disease is poor. The survival of patients with this disease is less than 5 years. It is probably best to become involved with an academic center in the area where research on interstitial lung diseases is studied in order to receive the latest treatments. These centers often are linked with a lung transplant program. Clinical trials are the best way of treating this disease at this time.

    What are causes and symptoms of pulmonary fibrosis?

    Symptoms of pulmonary fibrosis include:

    • shortness of breath,
    • coughing, and
    • diminished exercise tolerance.

    Symptoms vary depending on the cause of the pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.

    The most common form, idiopathic pulmonary fibrosis, has slow and relentless progression. Early on, patients often complain of a dry, unexplained cough. Often, slow and insidious onset of shortness of breath can set in. With time, dyspnea (shortness of breath) worsens. Dyspnea initially occurs only with activity and is often attributed to aging. Over time, the dyspnea occurs with little or no activity. Eventually, the shortness of breath becomes disabling, limiting all activity and even occurring while sitting still. In rarer cases, the fibrosis can be rapidly progressive, with dyspnea and disability occurring in weeks to months from the onset of the disease. This form of pulmonary fibrosis has been referred to as Hamman-Rich syndrome.

    How is pulmonary fibrosis diagnosed?

    Pulmonary fibrosis is suggested by a history of progressive (worsening over time) shortness of breath with exertion. Sometimes, during examination of the lungs with a stethoscope, the doctor can hear crackling sounds in the chest. These crackles have a very characteristic sound and are very similar to the sound heard when Velcro is pulled apart. These are often referred to as "Velcro crackles (or rales)". The chest X-ray may or may not be abnormal. However, a special X-ray test called a high resolution CT scan will frequently demonstrate abnormalities. This type of X-ray provides a cross-sectional picture of the lungs in very detailed resolution. The classic findings in idiopathic pulmonary fibrosis show diffuse peripheral scarring of the lungs with small bubbles (known as bullae) adjacent to the outer lining of the surface of the lung, often at the bases of the lungs.

    Lung function testing is distinctly abnormal. The volumes of the lungs may be reduced, as may the airflow, but the characteristic finding is a reduction in the diffusing capacity. The diffusing capacity is a measure of the ability of the lungs to exchange gases (oxygen and carbon dioxide) into and out of the blood stream.

    The diagnosis can be confirmed by lung biopsy. An open surgical biopsy, meaning that the chest wall must be surgically opened under general anesthesia to remove a portion of lung tissue, may be necessary to obtain enough tissue to make an accurate diagnosis. The most common type of biopsy in this situation is by a video assisted thoracoscope. Basically, this involves placing a small tube into the chest cavity through which biopsy samples can be obtained. Often, if the clinical situation is very classical in presentation, a biopsy may be unnecessary. The biopsy specimen is examined microscopically by a pathologist to confirm the presence of fibrosis.

    How is pulmonary fibrosis treated?

    The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition, since scarring is permanent once it has developed. Lung transplantation is the only therapeutic option available. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. Research trials using different drugs that may reduce fibrous scarring are ongoing.

    Since some types of lung fibrosis can respond to corticosteroids (such as prednisone)or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to decrease the processes that lead to fibrosis. These drugs do not help idiopathic pulmonary fibrosis. However, other causes of lung fibrosis may be responsive to immune suppression.

    The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent. Conditions that improve with immune suppressive treatment probably are not idiopathic pulmonary fibrosis.

    The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need and duration of treatment, and will monitor the response to therapy along with any side effects.

    Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids. These include

    • cyclophosphamide (Cytoxan),
    • azathioprine (Imuran, Azasan),
    • methotrexate (Rheumatrex, Trexall),
    • penicillamine (Cuprimine, Depen), and
    • cyclosporine.

    Learn more about: Cytoxan | Imuran | Azasan | Rheumatrex | Trexall | Cuprimine

    The anti-inflammatory medication colchicine has also been used with limited success. Other trials using drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone have not met with much success in the treatment of idiopathic pulmonary fibrosis.

    Learn more about: Cellcept

    Pulmonary fibrosis causes decreased oxygen levels in the blood. A decrease in blood oxygen level (hypoxia) can lead to elevated pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to failure of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are frequently treated with supplemental oxygen to prevent pulmonary hypertension. In some cases, new agents used to lower the blood pressure in the pulmonary artery have had limited benefit.

    There is also evidence that patients suffering from pulmonary fibrosis may be at increased risk for blood clots that travel to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be indicated.

    What are the complications of pulmonary fibrosis?

    Idiopathic pulmonary fibrosis tends to be relentless in its progression. The complications that occur are a reflection of the failure of the pulmonary system. Dyspnea, decreased activity, and signs of heart failure can occur. As the lungs fail, the blood pressure in the lungs rises. This results in increased work for and ultimately failure of the right side of the heart which pumps the blood through the lungs. This failure can result in fatigue, leg swelling, and overall fluid accumulation in the body. The immobility and sluggish blood flow can increase the risks for blood clots. Depression is frequently seen in this devastating disease.

    Can pulmonary fibrosis be prevented?

    Idiopathic pulmonary fibrosis has an increased frequency in cigarette smokers. This is just one more reason not to smoke. The cause of idiopathic pulmonary fibrosis is unknown, and therefore prevention is difficult. There is a rare form of idiopathic pulmonary fibrosis that runs in families. Work is being done at National Jewish Hospital in Denver, Colorado trying to identify markers for this disease.

    Unfortunately, since this is a fatal disease without effective therapy, there are many charlatans trying to take advantage of these stricken individuals and their families. There is no evidence that special diets or supplements or bowel preparations will help this disease in any way.

    What is the prognosis for pulmonary fibrosis?

    The prognosis of this disease is poor. The survival of patients with this disease is less than 5 years. It is probably best to become involved with an academic center in the area where research on interstitial lung diseases is studied in order to receive the latest treatments. These centers often are linked with a lung transplant program. Clinical trials are the best way of treating this disease at this time.

    Source: http://www.rxlist.com

    The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition, since scarring is permanent once it has developed. Lung transplantation is the only therapeutic option available. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. Research trials using different drugs that may reduce fibrous scarring are ongoing.

    Since some types of lung fibrosis can respond to corticosteroids (such as prednisone)or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to decrease the processes that lead to fibrosis. These drugs do not help idiopathic pulmonary fibrosis. However, other causes of lung fibrosis may be responsive to immune suppression.

    The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent. Conditions that improve with immune suppressive treatment probably are not idiopathic pulmonary fibrosis.

    The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need and duration of treatment, and will monitor the response to therapy along with any side effects.

    Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids. These include

    • cyclophosphamide (Cytoxan),
    • azathioprine (Imuran, Azasan),
    • methotrexate (Rheumatrex, Trexall),
    • penicillamine (Cuprimine, Depen), and
    • cyclosporine.

    Learn more about: Cytoxan | Imuran | Azasan | Rheumatrex | Trexall | Cuprimine

    The anti-inflammatory medication colchicine has also been used with limited success. Other trials using drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone have not met with much success in the treatment of idiopathic pulmonary fibrosis.

    Learn more about: Cellcept

    Pulmonary fibrosis causes decreased oxygen levels in the blood. A decrease in blood oxygen level (hypoxia) can lead to elevated pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to failure of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are frequently treated with supplemental oxygen to prevent pulmonary hypertension. In some cases, new agents used to lower the blood pressure in the pulmonary artery have had limited benefit.

    There is also evidence that patients suffering from pulmonary fibrosis may be at increased risk for blood clots that travel to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be indicated.

    Source: http://www.rxlist.com

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