Interstitial lung disease Flashcards
How do patients with interstitial lung disease often present ?
With progressive breathlessness,
a dry cough,
lung crackles
Diffuse infiltrates on CXR
What do lung function tests usually show
A restrictive pattern
–> normal ratio but decreased VC
An impaired gas diffusion
Hypoxaemia with hypocapnia
What is a classic example of a diffuse fibrotic lung disease
Idopathic pulmonary fibrosis
Does Iditiopathiic pulmonary fibrosis usually affect men or women
Men: 2:1
Do patients with Idiopathic pulmonary fibrosis have clubbing
Yes (60-70% do)
What causes idiopathic pulmonary fibrosis
Not entirely undertood but thought to be the result of a ffailyre of repair of lung tissuem whereby epithelial injury culminates in fibrosis rather than a controlled inflammatory and healing process
What do lung biospies of idiopathic pulmonary fibrosis show
usual interstitial pneumonia
Honeycombing
Where are the changes more severe
Subpleurally
What can show evidence of advanced fibrosis with extensive areas of reticulation and honeycombing
High resolution CT
What is a key feature of idiopathic pulmonary fibrosis on an Xray
Ground Glass opacities
What is the treatement for idiopathic pulmonary fibrosis
A combination of low-dose prednisolone, azathiprine and N-acetyl cysteine
What is the success rate of treatment of idiopathic pulmonary fibrosis
50% mortality within 3 years
What might be a treatment option for younger patients with idiopathic pulmonary fibrosis
Lung transplantation
What causes idiopathic pulmonary fibrosis
Not entirely understood but thought to be the result of a failure of repair of lung tissue whereby epithelial injury culminates in fibrosis rather than a controlled inflammatory and healing process
What is the treatment for idiopathic pulmonary fibrosis
A combination of low-dose prednisolone, azathiprine and N-acetyl cysteine
What might be a treatment option for younger patients with idiopathic pulmonary fibrosis
Lung transplantation