IPF Lung Disease
IPF stands for idiopathic pulmonary fibrosis, which is a type of interstitial lung disease (ILD). Interstitial lung disease causes inflammation in the airways that leads to scarring.
Idiopathic pulmonary fibrosis means the cause is not known. It involves scarring in the space and tissues that surround the air sacs in the lungs.
As IPF lung disease progresses, the inflammation and scarring become worse; the lungs become increasingly stiff and unable to get enough oxygen.
According to the American Lung Association, idiopathic pulmonary fibrosis is the most common form of ILD. In the United States, about 50,000 people are diagnosed with idiopathic pulmonary fibrosis each year.
Causes
Idiopathic refers to an unknown cause, which means it is not clear why this form of pulmonary fibrosis develops. But there are a few things that are known about the condition. According to the National Heart, Lung, and Blood Institute, more men than women are diagnosed with idiopathic pulmonary fibrosis. It is also most common in people in their 60s and 70s.
Risk Factors
Although the exact cause of idiopathic pulmonary fibrosis is not known, certain risk factors have been identified. Risk factors include the following:
Smoking: Smoking is a risk factor for most lung diseases, including idiopathic pulmonary fibrosis. People that smoke are at a higher risk of developing pulmonary fibrosis than nonsmokers.
Family history: Having a family history of idiopathic pulmonary fibrosis also increases your risk. Certain inherited genes have been identified as playing a role in the development of the disease.
History of acid reflux: Gastroesophageal reflux disease is associated with idiopathic pulmonary fibrosis. The theory is acid reflux may get into the lungs and lead to inflammation over time.
Air pollution: Exposure to air pollution and certain workplace substances may increase a person’s risk of pulmonary fibrosis.
Symptoms
Symptoms of idiopathic pulmonary fibrosis may be mild at first. Some people might not even realize they have the condition. As the disease progresses, symptoms typically increase in intensity. Symptoms include the following:
Shortness of breath: Shortness of breath may be one of the early symptoms of idiopathic pulmonary fibrosis. Shortness of breath typically becomes worse over time.
Coughing: Coughing with pulmonary fibrosis is usually nonproductive, which means it is a dry cough.
Painful joints and muscles: Joint and muscle pain may develop due to low oxygen levels. When breathing is impaired, it results in less oxygen into the lungs and subsequently throughout the body. The decrease in oxygen to the muscles and joints may lead to pain.
Fatigue: Idiopathic pulmonary fibrosis makes it hard to breathe. Increased work of breathing leads to fatigue. Shortness of breath and nighttime coughing can also make sleeping difficult, which also increases fatigue.
Idiopathic pulmonary fibrosis can also lead to complications, including pulmonary hypertension. Pulmonary hypertension develops when the pressure in the vessels that transport blood from the heart to the lungs is elevated beyond normal limits.
Treatment
Currently, there is no cure for idiopathic pulmonary fibrosis. But there are available treatments that may slow the progression of the disease and improve quality of life. Treatment may depend on the severity of the disease and may include the following:
Medications
Different classifications of medication are available to treat idiopathic pulmonary fibrosis. Two drugs classified as kinase inhibitors are approved by the FDA to slow the progression of the disease. The approved drugs include Nintedanib and Pirfenidone.
Nintedanib is an anti-fibrotic medication, which may slow the decline in lung function for people with idiopathic pulmonary fibrosis. Pirfenidone is also an anti-fibrotic as well as an anti-inflammatory medication. It may also help slow the progression of the condition. Both medications are taken by mouth and can have side effects. As with all medications, it is vital to weigh the risks versus the benefits.
Antacids may also be prescribed to treat acid reflux associated with pulmonary fibrosis. Although antacids will not treat fibrosis, it may prevent acid from getting into the lungs and making idiopathic pulmonary fibrosis worse.
Oxygen Therapy
Idiopathic pulmonary fibrosis makes it difficult to get oxygen into the lungs. This can lead to low oxygen levels in the body. Decreased oxygen levels may increase symptoms, such as fatigue and shortness of breath. Using supplemental oxygen won’t cure pulmonary fibrosis, but it may improve functioning. Various oxygen devices are used for home oxygen, including portable oxygen concentrators.
Lung Transplantation
Lung transplantation involves receiving lungs from a donor. It is a very involved process. Not everyone with pulmonary fibrosis is a good candidate for transplant. For instance, candidates must be in good physical health except for their lung disease to qualify.
Prior to being put on the lung transplant list, a candidate must go through a rigorous testing and screening process to make sure they are physically and emotionally ready for a lung transplant.
After qualifying for a transplant, the recipient waits for an appropriate match. Recovery after a lung transplant often involves taking medications for the rest of a person’s life to avoid rejecting the new lungs. According to research in the peer-reviewed journal Medical Sciences, the mean post lung transplant survival for people with pulmonary fibrosis is six years.