Idiopathic Pulmonary Fibrosis (IPF) is a type of chronic lung disease that occurs when tissues in the lungs become scarred and damaged. Over time, the scarring (called “fibrosis”), gets worse and it becomes progressively more difficult to breathe. IPF is an interstitial lung disease that affects the area around the air sacs of the lungs, known as the interstitium. Around 50,000 people in the United States have the disease, with approximately 15,000 new cases diagnosed annually.

Need to know IPF facts include the following:

1. Causes

Researchers do not yet know what exactly causes pulmonary fibrosis, hence the word “idiopathic,” which means “of unknown cause.” While the main cause is a mystery, there are certain risk factors will affect your chances, such as genetics. More than 20% of IPF patients have a relative who has an interstitial lung disease. Other risks include cigarette smoking, acid reflux, prolonged exposure to fumes and dust, as well as age and gender. 

2. Symptoms

There are two common symptoms of IPF: breathlessness and a persistent cough. The former is characterized by shortness of breath to the point that getting a full breath becomes difficult. People report that simple activities such as taking a shower or getting dressed can leave them out of breath. As the scarring worsens, patients report breathlessness even while resting. The second sign is a persistent cough that lasts longer than eight-weeks. This is described as a dry cough, but oftentimes is accompanied by mucus or phlegm. Other symptoms may include: sudden weight loss, loss of appetite, chest pain or tightness, lack of energy, clubbing of the fingers (widening and rounding of the fingertips), leg swelling. 

3. Treatments

The unknown cause of this disease makes it difficult for researchers to determine if there is a cure for IPF. While the scarring in the lungs can be slowed down, it cannot be reversed. With the help of your doctor, you can reduce your symptoms, prevent acute exacerbations, and prolong your life. Some treatments that you may want to discuss with your doctor include: oxygen therapy, ventilator support, and medications such as a kinase inhibitor or an antacid. In some cases, lung transplant surgery could be suggested. It is also important for IPF patients to keep current with immunizations to prevent pneumonia and influenza, which can exacerbate the symptoms.