Pulmonary fibrosis (PF) is a disease of the respiratory system caused by scarring (fibrosis) in the lungs, which makes it harder for patients to breathe. Based on the underlying cause of the scarring, PF can be grouped into several types.
Idiopathic pulmonary fibrosis, or IPF, is the most common form of the disease, estimated to affect one in every 5,000 people worldwide. In IPF, the underlying cause of fibrosis is not clear, hence the designation “idiopathic,” which means “of unknown cause.”
Like other forms of PF, IPF is a progressive disease, which means that symptoms are milder at the disease’s start and gradually become more severe with time.
Risk factors for IPF
While the specific cause of IPF is unknown, a variety of factors can influence a person’s risk of developing the disease. These include biological factors like genetics and other diseases, as well as environmental factors.
One of the most well-established risk factors for IPF is smoking. Other substances that irritate the lungs, such as agricultural by-products, wood or metal dusts, may also increase the risk of IPF.
Older age is also associated with a higher risk of IPF, as is male sex. Variations in the lung microbiome — the bacteria and other microorganisms that live in the lungs — can also affect the development of IPF.
While IPF is not a heritable disease, meaning that it is not passed from parents to their biological children, certain genetic variations may increase the risk a person has of developing the condition. Specific genes that have been tied to IPF include those involved in cellular aging (e.g., TERT and TERC), immune system activity (e.g., MUC5B and TOLLIP), and the lungs’ ability to bring in air (e.g., SFTPC, SFTPA1, and SFTPA2).
Other health conditions, including gastroesophageal reflux (acid reflux), diabetes, obstructive sleep apnea, and herpes, have also been linked to a higher risk of IPF.
Symptoms and diagnosis
Symptoms of IPF are broadly similar to those of other forms of PF. Shortness of breath is a common symptom, as is a dry, hacking cough. Swelling of the tips of the fingers and toes — a condition known as digital clubbing — is thought to be present in roughly 50% of IPF patients, and to be linked to a poorer prognosis.
Because IPF symptoms are generally similar to those of other respiratory diseases, IPF can be difficult to diagnose. A variety of diagnostic tests are available to detect scarring in the lungs, and to help distinguish IPF from other illnesses with similar symptoms.
Although no cure exists for IPF, the U.S. Food and Drug Administration (FDA) has approved two antifibrotic medications to treat the disorder: Esbriet (pirfenidone) and Ofev (nintedanib). In clinical trials, both of these therapies were shown to slow the lung function decline that marks IPF.
Oxygen therapy can also be used to help patients breathe easier and to improve their overall quality of life. Pulmonary rehabilitation programs provide a series of resources to help patients learn how to better manage and cope with their disease, and can used as a complement to pharmacological therapies.
In more severe cases, a lung transplant might be necessary. The process of getting a transplant can be arduous, and can include remaining on an organ donation waiting list for an extended period of time. People who receive an organ transplant generally need to take medications to suppress the immune system for the rest of their life, to reduce the risk of transplant rejection. Despite the risks and the considerable strain it poses on the body, a lung transplant may significantly prolong the life expectancy of people with severe IPF.
Last updated: Oct. 28, 2021
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