Interstitial Lung Disease

Question 1

What is fibrosis?

Answer 1

• a repair mechanism resulting in aberrant wound healing response
• responsible for prolongation of reparatory process

Question 2

What is the role of the interstitial compartment of the lungs?

Answer 2

• gas exchange
• antigen presenting

Question 3

What is traction bronchiectasis?

Answer 3

Airway dilatation associated with fibrosis

Question 4

How does fibrosis affect the lungs?

Answer 4

• makes lung smaller and stiffer
• decreased compliance
• peak flow = decreased total lung capacity and residual volume
• spirometry = decreased vital capacity, normal/increased FEV1/FVC
• decreased PaO2 and increased A-a O2 gradient (V/Q mis-match), PaCO2 normal

Question 5

What is the difference between inflammatory and fibrotic interstitial lung disease?

Answer 5

• inflammatory = reversible
• fibrotic = progressive

Question 6

List the factors associated with IPF

Answer 6

• smoking (current/ex)
• sex (male)
• heart burn
• age (esp >75)
• genetics (rare)

Question 7

What are the clinical features of IPF

Answer 7

• dyspnoea (SOB on exertion)
• dry cough
• progression over months/years
• clubbing
• CXR shows diffuse infiltrate

Question 8

What is the treatment for inflammatory interstitial lung disease?

Answer 8

• immunosuppressants
• eg. Steroids, azathioprine

Question 9

What is the treatment for fibrotic interstitial pulmonary disease?

Answer 9

• anti-fibrotic drugs
• eg. Pirfenidone, nintedanib