What is Idiopathic pulmonary fibrosis?

a chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs when no underlying cause exists

What patient groups is Idiopathic pulmonary fibrosis most common?

• aged 50-70 years
• men (2:1)

What are the main clinical features of Idiopathic pulmonary fibrosis?

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

What is the management of Idiopathic pulmonary fibrosis?

• pulmonary rehabilitation
• pirfenidone can benifit some patients
• supplementary oxygen
• eventually a lung transplant

What is the prognosis of Idiopathic pulmonary fibrosis?

poor, average life expectancy is around 3-4 years

What is seen on spirometry in Idiopathic pulmonary fibrosis?

classically a restrictive picture

What is seen on Gas transfer test (TLCO) in Idiopathic pulmonary fibrosis?

reduced

How is Idiopathic pulmonary fibrosis diagnosed?

Via CT thorax - showing bilateral interstitial shadowing