Idiopathic Pulmonary Fibrosis Flashcards
What does the term pulmonary fibrosis encompass?
Spectrum of diseases with multiple aetiologies
What is idiopathic pulmonary fibrosis (IPF)?
Progressive scarring and fibrosis of lung intersticium
As an aside (cus its not idiopathic), what drugs can cause pulmonary fibrosis?
- Methotrexate
- Bleomycin
- Amiodarone
What proportion of people presenting with IPF are over 60?
2/3
What causes IPF?
Unknown, but smoking has been implicated in its development
What is the pathophysiology of IPF?
Exact mechanism is unknown, but characteristic fibrosis and inflammation take place secondary to cytokine activation and mediation, and the normal tissue repair process if adversely affected
What is formed in the interstitium in IPF?
Fibroblastic foci
What does the formation of fibroblastic foci in the intersticium in IPF lead to?
Disruption of normal lung parenchyma and subsequent decrease in function
What are the clinical features of IPF?
- Persistent, progressive dyspnoea that is worse on exertion
- Cough
- Finger clubbing
- Bilateral inspiratory crackles on auscultation
What is found on PFTs in IPF?
Restrictive type pattern
What investigations should be done in IPF?
- Blood tests
- PFTs and gas transfer
- Review CXRs
- Perform high-res CT of thorax
What is found on CXR in IPF?
Bilateral lower zone reticulo-nodular shadow
What is the gold standard investigation in IPF?
High-resolution CT of the thorax
What is found on HRCT in IPF?
Ground glass appearance
What are the general principles in management of idiopathic pulmonary fibrosis?
- Pulmonary rehabilitation
- Supportive care
