PULMONARY FIBROSIS

Question 1

Is pulmonary fibrosis obstructive or restrictive?

Answer 1

Restrictive

Question 2

What is pulmonary fibrosis?

Answer 2

Group of diseases which produce interstitial lung damage and ultimately fibrosis and loss of elasticity of the lungs

Question 3

What are the three types?

Answer 3

o Replacement fibrosis – secondary to lung damage
o Focal fibrosis – in response to irritants
o Diffuse parenchymal lung disease

Question 4

What causes it?

Answer 4

May be secondary to a wide range of diseases or idiopathic

Question 5

What is the presentation like? (onset)

Answer 5

Can be acute, subacute or chronic

Question 6

What are idiopathic causes?

Answer 6

In some patients, the disease is genetic. Environmental factors (particularly exposure to certain types of dusts) may also play a role

Question 7

What happens over time?

Answer 7

mild scarring of the lung tissue occurs first, but, over months and years, the normal lung tissue is replaced by more heavily scarred lung tissue, which makes it difficult to breathe and deliver needed oxygen to the body.

Question 8

What are the risk factors?

Answer 8

• Viral Infections.
• Environmental agents:
• Exposure to dust / fumes and smoking
• (asbestos, silica, exposure to certain gases)
• Exposure to ionizing radiation (such as radiation therapy to treat tumors of the chest)
• Age /gender (diagnosis is more common in men and older people).
• Acid reflux from the stomach into the lungs.
• Chronic inflammatory processes (sarcoidosis, Wegener’s granulomatosis)
• Chronic conditions (lupus, rheumatoid arthritis)
• Certain medications

Question 9

What are the symptoms?

Answer 9

• Dry cough (can be chronic, dry, hacking coughing).
• SOB, especially during or after physical activity.
• Lasting tiredness.
• Weight loss.
• Clubbing

Question 10

What do you hear upon auscultation?

Answer 10

Fine crackles

Question 11

What investigations would you do?

Answer 11

• Blood tests to rule out other diseases.
• Pulmonary function test to measure breathing capacity.
• X-ray and CT (“CAT”) scan of your chest to identify the pattern of scarring in your lungs.
• Lung cell sample by passing a small flexible telescope (a bronchoscope) down the breathing tubes in order to examine cells and look for signs of inflammation.
• Lung biopsy.

Question 12

Is there a definitive treatment?

Answer 12

Uncurable

Pulmonary intervention and Oxygen therapy

Question 13

Living with pulmonary fibrosis: what advice would you give to patients?

Answer 13

• Quit smoking to help increase oxygen levels in your blood and lower blood pressure and heart rate.
• Eat less saturated fat and more fruits and vegetables.
• Exercise to help keep your body working as efficiently as possible.
• Lose excess weight to help improve your breathing capacity.
• Avoid situations in which you might catch infections from others.
• Avoid excessive pollutants and dust.
• Try to stay mentally active.
• Seek emotional support through family, friends, healthcare providers and support groups.
• Don’t over exert yourself and ensure you get enough rest