Idiopathic Pulmonary Fibrosis (ILD)

Question 1

What is Idiopathic Pulmonary Fibrosis?

Answer 1

Idiopathic interstitial pneumonia associated with inflammatory cell infiltrate and fibrosis

Question 2

What are the symptoms of Idiopathic Pulmonary Fibrosis?

Answer 2

Dry cough, exceptional SOB, malaise, weight loss and fatigue

Question 3

What are the signs of Idiopathic Pulmonary Fibrosis?

Answer 3

Clubbing & Bibasal, fine end inspiratory crackles

Question 4

What investigations can be used to confirm a diagnosis of Idiopathic Pulmonary Fibrosis?

Answer 4

Spirometry, DCLO, ABG & CXR

Question 5

What spirometry results would be indicative of Idiopathic Pulmonary Fibrosis?

Answer 5

Decreased FEV1, decreased FVC, normal FER (restrictive)

Question 6

What DCLO results would be indicative of Idiopathic Pulmonary Fibrosis?

Answer 6

Reduced

Question 7

What ABG results would be indicative of Idiopathic Pulmonary Fibrosis?

Answer 7

Reduced Pa02 / increased PaC02

Question 8

What CXR results would be indicative of Idiopathic Pulmonary Fibrosis?

Answer 8

Lower zone fibrosis

Question 9

What is the general management strategy of Idiopathic Pulmonary Fibrosis?

Answer 9

Smoking cessation, pulmonary rehab, oxygen and anti fibrotic agent (Perfenidone)

Question 10

What is the general prognosis of Idiopathic Pulmonary Fibrosis?

Answer 10

Not much can be done and 50% will die within 5 years