Pulmonary fibrosis

Question 1

What occurs during pulmonary fibrosis?

Answer 1

Infiltration of interstitial space and alveoli by:

• Inflammation factors
• Extracellular matrix and collagen
• Fibroblasts

Question 2

What are some of the presentation of pulmonary fibrosis?

Answer 2

Progressive exertion dyspnea over several months, unproductive cough/ sometimes productive (clear or white)

Question 3

What are some signs of pulmonary fibrosis?

Answer 3

Clubbing, bilateral reduced chest expansion, fine late inspiratory ‘velcro’ crepitations bibasal, no changes to percussion or resonance,

Question 4

What are the investigations for pulmonary fibrosis and the findings?

Answer 4

• X-ray: Reticulonodular infiltrates, bilateral, may be normal in early stages
• Lung function test: Restrictive profile and reduced diffusion capacity
• *CT scan:
• Blood test: Rule out rheumatoid and immunological causes (i.e. ANCA)

Question 5

What are the two subtypes of pulmonary fibrosis?

Answer 5

NSIP (inflammatory cause) and UIP (fibrotic disease)

Question 6

How is pulmonary fibrosis treated?

Answer 6

No treatment to reverse fibrosis.
NSIP - Responds more to treatment, systemic corticosteroids, immunosuppressants (e.g. cyclophosphamide)
UIP - Antifibrolytics (e.g. pirfenidone), N-acetylcysteine
Oxygen therapy, pulmonary rehabilitation, diuretics for pulmonary oedema