What is Pulmonary Fibrosis?

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.