Learn about the disease, illness and/or condition Pulmonary Fibrosis including: symptoms, causes, treatments, contraindications and conditions at ClusterMed.info.
Pulmonary Fibrosis Information
Pulmonary fibrosis definition and facts
Can pulmonary fibrosis be prevented?
Idiopathic pulmonary fibrosis has an increased frequency in cigarette smokers. The cause of idiopathic pulmonary fibrosis is unknown, and therefore prevention is difficult. There is a rare form of idiopathic pulmonary fibrosis is hereditary (runs in families). Work is being done at National Jewish Hospital in Denver, CO 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.
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 of pulmonary fibrosis 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. 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.
What about pulmonary rehabilitation for pulmonary fibrosis?
Pulmonary rehabilitation along with medical care helps improve the quality of life of people who have chronic breathing problems such as pulmonary fibrosis. It involves:
What are signs and symptoms of pulmonary fibrosis?
Signs and symptoms of pulmonary fibrosis include:
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. Shortness of breath, 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.
What are the signs and symptoms of idiopathic pulmonary fibrosis?
The most common form, idiopathic pulmonary fibrosis, has a slow and relentless progression.
What are the stages of idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis has been loosely staged and categorized as mild, moderate, or severe, or early versus advanced. The staging is usually based on pulmonary function tests, although these stages are considered arbitrary. There are some newer proposed staging systems:
What causes pulmonary fibrosis?
Pulmonary fibrosis can be caused by many conditions including:
What is idiopathic pulmonary fibrosis?
The definition of "idiopathic" is "of unknown cause;" thus idiopathic pulmonary fibrosis (IPF) is fibrosis (scarring) of the lungs without a known cause.IPF mostly affects middle-aged and older adults, and there is no cure. The progress of the disease varies from person to person, but often people diagnosed with idiopathic pulmonary fibrosis live about three to five years following their diagnosis. While the cause of IPF is unknown, it is thought that genetics may be a factor. When more than one member of a family has IPF, the disease is referred to as familial idiopathic pulmonary fibrosis.
What is pulmonary fibrosis?
"Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs.
What is the prognosis and life expectancy for a person with pulmonary fibrosis?
The prognosis of this disease is poor. The survival of patients with pulmonary fibrosis is less than 5 years. It is 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 is the treatment for pulmonary fibrosis?
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. Two drugs, nintedanib (Ofev) and pirfenidone (Esbriet, pirfenex, Pirespa) have offered some new hope. The results from two large studies show some slowing of progression of fibrosis, as well as some minor changes to tests of lung function. Unfortunately, the side effects from both of these drugs have resulted in some patients being unable to take this medication. 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 sometimes are 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 especially if a different cause for interstitial lung disease is suspected. These include:
Where can I get information to improve the quality of life for those with pulmonary fibrosis?
For information quality of life and living with pulmonary fibrosis please visit Pulmonaryfibrosis.org.
Which specialties of doctors treat pulmonary fibrosis?
If you have not been diagnosed with pulmonary fibrosis, but have any symptoms of the disease such as shortness of breath, chronic dry cough, diminished exercise tolerance, shallow breathing, chest discomfort, weakness, or other symptoms, see a doctor. If you have already been diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis you should be monitored regularly, and you should referred for evaluation for lung transplantation, even if one is not needed at the immediate time.
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