What Is Idiopathic Pulmonary Fibrosis (IPF)?

Diana J. Smith

Idiopathic pulmonary fibrosis is an uncommon and frequently fatal lung disease that’s difficult to diagnose. More research—and awareness—is needed to improve outcomes.

IPF is a disease that causes scarring (fibrosis) of the lungs. It’s the most common type of pulmonary fibrosis, a group of serious lung diseases that affect the respiratory system. The word “idiopathic” means it has no known cause. Scarring causes stiffness in the lungs and makes it difficult to breathe.

Lung damage from IPF is irreversible and progressive, meaning it gets worse over time. And the rate of progression can vary greatly from one person to another. In some cases, it can be slowed by certain medications. Occasionally, people with IPF will be recommended for lung transplant. But ultimately, IPF results in life-threatening complications such as respiratory failure.

The symptoms of IPF make this disease difficult to diagnose as they are nonspecific. Symptoms can range from being asymptomatic to having a chronic dry cough, shortness of breath, and/or fatigue. Because symptoms are similar to other illnesses, like the common cold, or may appear mild or absent early on, many patients are not diagnosed until the disease progresses to its later stages

According to the National Institutes of Health NIH, about 100,000 people in the U.S. have IPF. Approximately 30,000 to 40,000 new cases are diagnosed each year. More than 50,000 people die from IPF in the U.S. each year, more deaths than from breast cancer. It is more common in men than women, and usually affects people between the ages of 50 and 70.

While studies show that Blacks are less common than Whites to develop IPF, death from the disease occurs at a younger age in those of African descent, according to this study. Earlier onset and worsened outcomes in Blacks might prompt the need for earlier therapeutic intervention.

Although there is no known cause for IPF, studies show that there are certain factors that increase the risk of receiving an IPF diagnosis, according to the Pulmonary Fibrosis Foundation. Current and former smokers are more likely to develop IPF than those who have never smoked.

A family history of pulmonary fibrosis is also a risk factor, as are certain genes. Some evidence suggests that certain viral infections, air pollution, and some exposures in the workplace may also be risk factors for IPF.

There are also conditions, such as gastroesophageal reflux disease (acid reflux, heartburn, or GERD , sleep apnea, or pulmonary hypertension that are often present in people who are diagnosed with IPF.  The presence of risk factors and comorbidities provide clues that may be helpful to a physician who suspects IPF.

When a doctor or other healthcare provider suspects IPF, they will collect information about the patient’s medical and personal history, work and home environment, hobbies, and illness that may be present in the family. This can help a doctor identify exposures or other diseases that might have caused lung injury and scarring. The doctor will also often order pulmonary function tests, a chest x-ray, blood work, and a high-resolution CT scan.

However, misdiagnosis and delays in diagnosis of IPF are common. In one study, IPF was most often misdiagnosed as asthma 13.5% , pneumonia 13.0% , or bronchitis 12.3% . Delays in diagnosis have been reported to be from one year to as long as three years, with longer delays associated with an increased risk of death.

Some people live only months after a pulmonary fibrosis diagnosis. Others live several years. Many factors affect a patient’s prognosis. Even medical providers can’t predict some of these factors.

Overall, there is relatively little research into IPF, an under-recognized disease, according to the IPF Foundation. That’s why participation in clinical studies by people living with IPF is critical so that more can be learned about the causes of pulmonary fibrosis and find new treatments for IPF.

FibroGen, Inc’s ZEPHYRUS Phase 3 clinical study aims to slow progression of IPF via pamrevlumab, an antibody designed to bind to and block the activity of connective tissue growth factor CTGF .

There is growing evidence that CTGF plays a causal role in the progressive lung scarring that is characteristic of IPF.)

If you’ve been diagnosed with IPF, you can take steps to help your body stay in its best possible shape. Be proactive to avoid getting sick. Keep up-to-date with vaccines, as lung scarring makes it harder for the body to fight against infections. Stay active, make smart food choices, get plenty of rest—and don’t smoke.

 

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