Idiopathic pulmonary fibrosis

Question 1

Cause

Answer 1

IPF is reserved when no underlying cause exists

Question 2

Epidemiology

Answer 2

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

Question 3

Features

progressive […] dyspnoea
[…] […] end-[…] crepitations on auscultation
[…] cough
clubbing

Answer 3

Features

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

Question 4

Diagnosis

• […] picture (FEV1 […]/decreased, FVC […], FEV1/FVC […])
• impaired gas exchange: reduced […] […] ([…])
• imaging: bilateral […] […] (typically small, irregular, peripheral […] - ‘[…]-[…]’ - later progressing to ‘[…]’) may be seen on a chest x-ray but […]-[…] […] is the investigation of choice and required to make a diagnosis of IPF

Answer 4

Diagnosis

restrictive 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 but high-resolution CT scanning is the investigation of choice and required to make a diagnosis of IPF

Question 5

Mx of IPF

Answer 5

Pulmonary rehabilitation
Pirfenidone
LTOT
Lung transplant

Question 6

Prognosis

Answer 6

Average life expectancy is around 3-4 years