Idiopathic pulmonary fibrosis

Question 1

What is Idiopathic Pulmonary Fibrosis (IPF)?

Answer 1

chronic lung condition characterized by progressive fibrosis of the lung interstitium with no known underlying cause.

Question 2

What are the common features of IPF?

Answer 2

progressive exertional dyspnoea,
bibasal fine end-inspiratory crepitations,
dry cough
clubbing.

Question 3

What is the typical age range for patients with IPF?

Answer 3

typically seen in patients aged 50-70 years and is twice as common in men.

Question 4

What are the findings on spirometry in IPF?

Answer 4

Restrictive pattern:
FEV1: Normal or decreased
FVC: Decreased
FEV1/FVC: Increased

Question 5

What is the most common imaging finding in IPF?

Answer 5

Bilateral interstitial shadowing
Ground-glass opacities (early)
Honeycombing (later)
High-resolution CT is diagnostic

Question 6

What is the role of high-resolution CT in diagnosing IPF?

Answer 6

High-resolution CT is the investigation of choice to diagnose IPF and helps visualize characteristic patterns like ground-glass opacities and honeycombing.

Question 7

What are the typical autoimmune markers seen in IPF?

Answer 7

ANA is positive in 30% of cases, and rheumatoid factor is positive in 10%.

However, these do not necessarily indicate a secondary connective tissue disease.

Question 8

What is the management approach for IPF?

Answer 8

Management includes pulmonary rehabilitation, the potential use of pirfenidone (an antifibrotic agent) in selected patients, oxygen therapy, and lung transplant in advanced cases.

Question 9

What is the average life expectancy after diagnosis of IPF?

Answer 9

3-4 years (poor prognosis).