Idiopathic pulmonary fibrosis

Question 1

What is IPF?

Answer 1

a chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs. Whilst there are many causes of lung fibrosis (e.g. medications, connective tissue disease, asbestos) the term IPF is reserved when no underlying cause exists.

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

Question 2

Features of IPF?

Answer 2

• progressive exertional dyspnoea
• bibasal fine end-inspiratory crepitations on auscultation
• dry cough
• clubbing

Question 3

Diagnosis of IPF?

Answer 3

• spirometry: classically arestrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)
• impaired gas exchange: reduced transfer factor (TLCO)
• imaging: bilateral interstitial shadowing (typically small, irregular, peripheral opacities - ‘ground-glass’ - later progressing to ‘honeycombing’) may be seen on a chest x-ray buthigh-resolution CT scanning is the investigation of choiceand required to make a diagnosis of IPF
• ANA positive in 30%, rheumatoid factor positive in 10% but this does not necessarily mean that the fibrosis is secondary to a connective tissue disease. Titres are usually low

Question 4

Management of IPF

Answer 4

• pulmonary rehabilitation
• very few medications have been shown to give any benefit in IPF. There is some evidence thatpirfenidone (an antifibrotic agent) may be useful in selected patients(see NICE guidelines)
• many patients will require supplementary oxygen and eventually a lung transplant

Question 5

Prognosis of IPF?

Answer 5

poor, average life expectancy is around 3-4 years

Question 6

What may a CT scan show?

Answer 6

Advanced pulmonary fibrosis can show ‘honeycombing’

traction bronchiectasis