Idiopathic Pulmonary Fibrosis

Question 1

There are many causes of lung fibrosis, but what makes them different from IPF?

Answer 1

In IPF no cause can be found.

Question 2

In what gender is IPF more common?

Answer 2

Men

Question 3

In what age group is IPF most commonly found?

Answer 3

50-70 years old

Question 4

How do you diagnose IPF?

Answer 4

• Spirometry: classically a 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
• 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 5

What is the prognosis of IPF?

Answer 5

Poor

Average life expectancy is 3-4 years after diagnosis.

Question 6

What is the management plan for IPF?

Answer 6

• Pulmonary rehabilitation
• Very few medications have been shown to give any benefit in IPF. There is some evidence that pirfenidone (an antifibrotic agent) may be useful in selected patients.
• Many patients will require supplementary oxygen and eventually a lung transplant.