What is pulmonary fibrosis?

The word “pulmonary” means lung and the word “fibrosis” means scar tissue— similar to scars that form on the skin from an old injury or surgery. Pulmonary fibrosis (PF) is a process that causes lung scarring, in which fibrotic tissue blocks the movement of oxygen from inside the tiny air sacs in the lungs into the bloodstream. Low oxygen levels (and the stiff scar tissue itself) can cause people who have pulmonary fibrosis to feel short of breath, particularly when walking and exercising.

No one is certain how many people are affected by PF. One recent study estimated that idiopathic pulmonary fibrosis (or IPF, which is just one of more than 200 types of PF) affects 1 out of 200 adults over the age of 70 in the United States. There are over 250,000 Americans living with PF today. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 Americans die from IPF each year.

The clinical course of PF is highly variable and often difficult to predict. It is important for a PF patient to have an accurate diagnosis because their treatments, disease management and prognosis may vary depending on the type of pulmonary fibrosis they have. Even among patients with similar diagnoses, some may experience rapid progression of their disease while others can remain at the same level of functioning for a longer time. Many types of PF can be complicated by episodes of sudden declines in lung function and oxygen levels, known as acute exacerbation, from which most patients do not fully recover. These events can sometimes result in other problems such as heart failure, high blood pressure in the lungs (pulmonary hypertension) and other co-morbidities (diseases).

As the lung scarring in PF progresses, breathing becomes more difficult with increased demand for higher flows of oxygen. This demand for more oxygen may cause increased breathlessness, particularly with activity, and anxiety. Patients may also experience an increase in other associated symptoms as their disease progresses such as cough, fatigue, and deconditioning.