• Slide 1 of 16: The lungs are made up of hundreds of millions of tiny, delicate air sacs called alveoli, which play a vital role in distributing oxygen to the body through the bloodstream. Healthline calls them the “workhorses of your respiratory system,” because they are responsible for providing the body with fresh air and expelling harmful carbon dioxide. With pulmonary fibrosis, these sacs begin to scar and stiffen, making it difficult for them to do their job. Here’s everything you need to know about idiopathic pulmonary fibrosis, including the causes, symptoms, and treatments.
  • Slide 2 of 16: Pulmonary fibrosis is a chronic and progressive lung disease that causes scar tissue to accumulate in the lungs, affecting their ability to transport oxygen to the body through the bloodstream. Idiopathic refers to a form of the disease in which the cause is unknown.

    What is IPF?

    Pulmonary fibrosis is a chronic and progressive lung disease that causes scar tissue to accumulate in the lungs, affecting their ability to transport oxygen to the body through the bloodstream. Idiopathic refers to a form of the disease in which the cause is unknown.

  • Slide 3 of 16: While the exact cause of the disease remains unknown, fibrosis in the lungs is believed to be the result of abnormal tissue repair in response to tissue damage. External factors are also suspected to play a role in the development of IPF. These factors include:Cigarette smokingExposure to wood or metal dustViral infectionsCertain medications

    What causes it?

    While the exact cause of the disease remains unknown, fibrosis in the lungs is believed to be the result of abnormal tissue repair in response to tissue damage. External factors are also suspected to play a role in the development of IPF. These factors include:

    • Cigarette smoking
    • Exposure to wood or metal dust
    • Viral infections
    • Certain medications
  • Slide 4 of 16: Familial pulmonary fibrosis (FPF) is when two or more family members have IPF. As MedlinePlus notes, FPF is far less common than the sporadic form of the disease, with only a small percentage of IPF cases running in the family.

    Is it hereditary?

    Familial pulmonary fibrosis (FPF) is when two or more family members have IPF. As MedlinePlus notes, FPF is far less common than the sporadic form of the disease, with only a small percentage of IPF cases running in the family.

                  Who does it affect most?

Most people who develop IPF are between the ages of 50 and 70 (the mean age is 65-70). Men are also more prone to the disease than women. According to a 2021 study, patient numbers are on the rise around the world, likely due to the aging population, an increased awareness of the disease, and more effective diagnostic testing.

                 How common is it?

IPF is a rare, but serious, disease. According to MedlinePlus, roughly 100,000 Americans are currently living with it, while 30,000 to 40,000 new cases are discovered each year. Worldwide, it affects an estimated 13 to 20 of every 100,000 people.

What are the symptoms?

Common symptoms of IPF include:

  • Shortness of breath
  • Chronic, dry cough
  • Fatigue
  • Loss of appetite/weight loss
  • Clubbed fingertips and toesWhat are the complications?What are the complications?

    What are the complications?

    IPF has been linked to a number of co-morbidities, including lung cancer, emphysema, obstructive sleep apnea (OSA), gastroesophageal reflux disease (GERD), and multiple cardiovascular consequences. Scar tissue on the lungs can cause damage to the heart by restricting the flow of oxygen to the body. This may lead to high blood pressure in the lungs (known as pulmonary hypertension) or even heart failure.