What is pulmonary fibrosis?
"Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs.
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 causes pulmonary fibrosis?
Pulmonary fibrosis can be caused by many conditions including:
- Chronic inflammatory processes (sarcoidosis, Wegener's granulomatosis)
- 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)
- Certain medications
In a condition known as hypersensitivity pneumonitis, fibrosis of the lung can develop following a heightened immune reaction to inhaled organic dust 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 the symptoms of pulmonary fibrosis?
Signs and symptoms of pulmonary fibrosis include:
- Shortness of breath (dyspnea), including shortness of breath with everyday activities
- Coughing (chronic, dry, hacking cough)
- Diminished exercise tolerance
- Fast, shallow breathing
- Chest discomfort
- Muscle and joint aches
- Loss of appetite
- Unexplained/unintended weight loss
- "Clubbing" of the tips of the fingers and toes (thickening of flesh under the nails, causing a widening and rounding appearance
Symptoms vary depending on the cause of pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.
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Which specialties of doctors diagnose and 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 be referred for evaluation for lung transplantation, even if one is not needed at the immediate time.
How is pulmonary fibrosis diagnosed?
Pulmonary fibrosis is suggested by a history of progressive (worsening over time) shortness of breath with exertion. Sometimes, during the 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 bloodstream.
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.
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What are the stages of idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis has been loosely staged and categorized as mild, moderate, 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:
- Mortality risk scoring system
- This is based on four predictors:
- Recent respiratory hospitalization
- Baseline forced vital capacity (FVC, a measure of lung function)
- 24-week change in FVC
- This is based on four predictors:
Taking these factors into account gives a score, which helps predict the expected 1-year probability of death.
- The GAP index
- ???????This staging system is based on four baseline variables:
- Gender (G)
- Age (A)
- Two lung physiology variables (P) (FVC plus the diffusing capacity of the lung for carbon monoxide, or DLCO,)
- The GAP index uses these variables to categorize patients in three different stages, which estimate the individual risk for patients.
- ???????This staging system is based on four baseline variables:
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 the progression of fibrosis, as well as some minor changes to tests of lung function. Unfortunately, the side effects of 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:
- cyclophosphamide (Cytoxan)
- azathioprine (Imuran, Azasan)
- methotrexate (Rheumatrex, Trexall)
- penicillamine (Cuprimine, Depen)
The anti-inflammatory medication colchicine has also been used with limited success. Other trials using drugs such as gamma interferon and mycophenolate mofetil (Cellcept) have not met with much success in the treatment of idiopathic pulmonary fibrosis.
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 therapy to prevent pulmonary hypertension.
A consensus statement was issued revising the 2011 clinical practice guidelines for the treatment of idiopathic pulmonary fibrosis. This statement was issued by one of the largest respiratory physician groups in the world, including members from the United States, Europe, Japan, and Latin America. These recommendations are partially based on a recent study involving the use of prednisone, azathioprine, and N-acetylcysteine, the so-called triple therapy. In this study, drug recipients had a 10-fold increase in the death rate than the placebo group. These new guidelines therefore strongly discourage the use of triple therapy in idiopathic pulmonary fibrosis. These guidelines also suggest that anticoagulation and anti-GERD (gastroesophageal reflux disease) not be administered for idiopathic pulmonary fibrosis alone. These medications should not be used without other medical indications in patients with idiopathic pulmonary fibrosis. Lastly, patients with idiopathic pulmonary fibrosis often have pulmonary hypertension as their disease progresses. There is currently no indication to use pulmonary hypertensive medication in these patients except oxygen in those with low oxygen levels.
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:
- Exercise conditioning
- Nutrition counseling
- Patient education on managing the disease
- Breathing strategies
- Techniques to help conserve energy
- Counseling and/or support groups
Patients diagnosed with idiopathic pulmonary fibrosis who are current smokers are encouraged to quit. It is also recommended that patients with IPF receive vaccination against influenza and pneumococcal infection.
What is the prognosis for 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 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 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. Immobility and sluggish blood flow can increase the risks of 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. The cause of idiopathic pulmonary fibrosis is unknown, and therefore prevention is difficult. There is a rare form of idiopathic pulmonary fibrosis that 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, many charlatans are 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.
Where can I get information to improve the quality of life for those with pulmonary fibrosis?
For information on the quality of life and living with pulmonary fibrosis please visit Pulmonaryfibrosis.org.
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Godfrey, M.K., MD. "Idiopathic Pulmonary Fibrosis Treatment & Management." Updated: Aug 11, 2016.
Kolb, M., et al. "Staging of idiopathic pulmonary fibrosis: past, present and future." European Respiratory Review 2014 23: 220-224; DOI: 10.1183/09059180.00002114
Nathan, S. MD. et al. "Long-term Course and Prognosis of Idiopathic Pulmonary Fibrosis in the New Millennium." Chest. 2011;140(1):221-229.
National Heart, Lung, and Blood Institute. What Is Idiopathic Pulmonary Fibrosis? Updated: Sep 20, 2011.
Pulmonary Fibrosis Foundation. "About PF."
Raghu. et al. "An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline" Am J resp crit care medicine abstract 2015; 192; e3-19.
Richeldi. et al. "Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis." N Engl J Med 2014; 370:2071-2082.
Talmadge. et al. "A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis." N Engl J Med 2014; 370:2083-2092.
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Pulmonary fibrosis is scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes, infections, environmental agents, exposure to ionizing radiation, chronic conditions, and certain medications. Symptoms include
- shortness of breath,
- coughing, and
- diminished exercise tolerance.
Treatment options are dependent on the type of pulmonary fibrosis; lung transplant and/or medications are options.
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