Idiopathic Pulmonary Fibrosis

Question 1

Define idiopathic pulmonary fibrosis

Answer 1

Chronic idiopathic inflammatory condition resulting in fibrosis of alveoli and interstitium

Question 2

What are the causes/risk factors of idiopathic pulmonary fibrosis?

Answer 2

• Smoking
• Male
• Chronic microaspiration
(GORD)
• Organic dust exposure e.g.
livestock farming
• Inorganic dust exposure
e.g. metal, wood, stone

Question 3

What are the presenting symptoms of idiopathic pulmonary fibrosis?

Answer 3

• Progressively worsening dyspnoea (on
exertion)
• Dry cough
• Weight loss
• Fatigue
• Malaise

Question 4

What are the signs of idiopathic pulmonary fibrosis?

Answer 4

• Fine inspiratory crackles “Velcro”
• Clubbing

Signs of right heart failure in advanced disease
• RV heave
• Raised JVP
• Peripheral oedema

Question 5

What are the investigations for idiopathic pulmonary fibrosis?

Answer 5

• CXR - basilar, peripheral, bilateral, asymmetrical, reticular opacities
• high res CT - basilar- and subpleural-predominant areas of increased reticulation, honeycombing, and possible traction bronchiectasis or bronchiolectasis
• anti-nuclear antibodies - normal or mildly elevated
• rheumatoid factor - normal or mildly elevated
• anti-cyclic citrullinated peptide - normal
• myositis panel - normal