UNDERSTANDING PULMONARY FIBROSIS
What is Pulmonary Fibrosis
Pulmonary fibrosis (PF) describes a condition in which the lung tissue becomes thickened, stiff, and scarred. The medical term used to describe this scar tissue is fibrosis. The air sacks and blood vessels within the lung are responsible for delivering oxygen to the body. As lung tissue becomes scarred and thickened, it is difficult for the lungs to transfer oxygen into the bloodstream. As a result, the brain, heart, and other organs don't get the oxygen they need to function properly. In some cases, doctors can determine the cause of the PF, but in most cases, the cause remains unknown. When there is no known cause for the development of PF (and certain criteria are met) the disease is called idiopathic pulmonary fibrosis or IPF.
Symptoms of Pulmonary Fibrosis
One reason pulmonary fibrosis often goes misdiagnosed is that symptoms of PF can be similar to symptoms of other lung diseases. Most people with PF develop symptoms between the ages of 50 and 70 years. If you have any of the symptoms below, talk with your doctor right away and be persistent if you feel your lung health is not improving. Also be sure to tell your doctor about any family history of lung disease or any exposure to risk factors you may have had. There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to make the right diagnosis.
Types of Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis (IPF)
The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. This means this type of PF has no known cause. Approximately 50,000 new cases of IPF are diagnosed each year. Most IPF patients first start noticing symptoms between the ages of 50 and 70 years old. It is more common in men, but the number of cases of IPF in women is on the rise.
PF from Diseases
Some cases of PF are caused by autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren's syndrome. Certain viral infections and gastroesophageal reflux disease (GERD) are also risk factors for PF. GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.
Familial PF
Familial PF is very rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or any other form of idiopathic interstitial pneumonia (IIP). There are genes that have been linked to PF but much is still unknown about this field.
PF from Exposures
PF can be caused by exposure to hazardous materials. Examples include occupational exposures such as asbestos or silica. Some cases of PF are caused by breathing in bird or animal droppings. Radiation treatments and certain types of medications can cause PF. Cigarette smoking also increases a person's risk of developing PF.
How is Pulmonary Fibrosis Diagnosed?
Symptoms: Discussing symptoms with your doctor is key to determining the severity of your PF. You might be asked about how frequently you become breathless or how often you cough. Worsening breathlessness and cough don't always mean the disease has progressed. Knowing your symptoms helps your doctor better understand your particular disease.
Pulmonary Function Tests (PFTs) or Lung Function Tests give your doctor important information about the amount of air the lungs can hold and how forcefully you can empty air from the lungs. A PFT reveals your forced vital capacity (FVC) which is the amount of air that is exhaled starting from a maximal inhalation. This percentage can help your physician understand if you have mild, moderate, severe or very severe PF. The most basic test is spirometry.
A six-minute walk test may be done to measure your exercise capacity. A high-resolution CT scan (HRCT) shows how much scarring is in the lungs.
Pulmonary fibrosis (PF) describes a condition in which the lung tissue becomes thickened, stiff, and scarred. The medical term used to describe this scar tissue is fibrosis. The air sacks and blood vessels within the lung are responsible for delivering oxygen to the body. As lung tissue becomes scarred and thickened, it is difficult for the lungs to transfer oxygen into the bloodstream. As a result, the brain, heart, and other organs don't get the oxygen they need to function properly. In some cases, doctors can determine the cause of the PF, but in most cases, the cause remains unknown. When there is no known cause for the development of PF (and certain criteria are met) the disease is called idiopathic pulmonary fibrosis or IPF.
Symptoms of Pulmonary Fibrosis
One reason pulmonary fibrosis often goes misdiagnosed is that symptoms of PF can be similar to symptoms of other lung diseases. Most people with PF develop symptoms between the ages of 50 and 70 years. If you have any of the symptoms below, talk with your doctor right away and be persistent if you feel your lung health is not improving. Also be sure to tell your doctor about any family history of lung disease or any exposure to risk factors you may have had. There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to make the right diagnosis.
- Shortness of breath, particularly during exercise
- Dry, hacking cough
- Fast, shallow breathing
- Gradual unintended weight loss
- Tiredness
- Aching joints and muscles
- Clubbing (widening and rounding) of the tips of the fingers or toes
Types of Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis (IPF)
The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. This means this type of PF has no known cause. Approximately 50,000 new cases of IPF are diagnosed each year. Most IPF patients first start noticing symptoms between the ages of 50 and 70 years old. It is more common in men, but the number of cases of IPF in women is on the rise.
PF from Diseases
Some cases of PF are caused by autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren's syndrome. Certain viral infections and gastroesophageal reflux disease (GERD) are also risk factors for PF. GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.
Familial PF
Familial PF is very rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or any other form of idiopathic interstitial pneumonia (IIP). There are genes that have been linked to PF but much is still unknown about this field.
PF from Exposures
PF can be caused by exposure to hazardous materials. Examples include occupational exposures such as asbestos or silica. Some cases of PF are caused by breathing in bird or animal droppings. Radiation treatments and certain types of medications can cause PF. Cigarette smoking also increases a person's risk of developing PF.
How is Pulmonary Fibrosis Diagnosed?
Symptoms: Discussing symptoms with your doctor is key to determining the severity of your PF. You might be asked about how frequently you become breathless or how often you cough. Worsening breathlessness and cough don't always mean the disease has progressed. Knowing your symptoms helps your doctor better understand your particular disease.
Pulmonary Function Tests (PFTs) or Lung Function Tests give your doctor important information about the amount of air the lungs can hold and how forcefully you can empty air from the lungs. A PFT reveals your forced vital capacity (FVC) which is the amount of air that is exhaled starting from a maximal inhalation. This percentage can help your physician understand if you have mild, moderate, severe or very severe PF. The most basic test is spirometry.
A six-minute walk test may be done to measure your exercise capacity. A high-resolution CT scan (HRCT) shows how much scarring is in the lungs.