What is Pulmonary Fibrosis?

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What is pulmonary fibrosis?

Pulmonary fibrosis (PF) is a type of lung disease that affects the interstitium of the lung (the space between the air sacs in the lung). There are many different types of pulmonary fibrosis, with different etiologies, treatments, and prognoses. In patients with PF, the lung tissue becomes scarred and stiff, and over time may become more widespread. In turn, the lungs lose their ability to transfer oxygen to the bloodstream, resulting in shortness of breath and vital organs being deprived of the necessary oxygen to survive.

What are the symptoms of pulmonary fibrosis?

Symptoms of pulmonary fibrosis usually have a gradual onset and may include:

  • Shortness of breath, particularly during or after physical activity
  • Spasmodic, dry cough
  • Gradual, unintended weight loss
  • Fatigue and weakness
  • Chest discomfort
  • Clubbing, or enlargement, of the ends of the fingers (or sometimes the toes) due to a buildup of tissue
What causes pulmonary fibrosis?

PF can be linked to particular causes, such as environmental exposures, medications, chemotherapy or radiation therapy, or autoimmune diseases such as scleroderma or rheumatoid arthritis. In many cases of pulmonary fibrosis, the cause is uncertain or unknown and as such is termed idiopathic.

Idiopathic Pulmonary Fibrosis: A form of PF

Download the UHN Patient Education handout – IPF Information for patients and families

When Robert Davidson was diagnosed with IPF, limited information about the disease inspired him to create the Canadian Pulmonary Fibrosis Foundation (CPFF) as an important resource for people living with IPF.