Pulmonary fibrosis Flashcards
What are the 3 main mechanisms of pathophysiology of Pulmonary Fibrosis?
- Injury - damage to endothelial cells releases inflammatory mediators
- Inflammation - recruit inflammatory cells and release chemokines
- Repair - cellular reorganisation gone awry
What is ‘Interstitial Pneumonitis’?
- Excessive extracellular matrix deposition
- Fibroblast and myofibroblast accumulate between the vascular and alveolar endothelium
- Results in ‘honeycomb appearance’
Name 3 features of a Chest X ray in Pulmonary fibrosis?
- Increased interstitial marking
- Reduced lung volumes
- bilateral interstitial shadowing (typically small, irregular, peripheral opacities - ‘ground-glass’ - later progressing to ‘honeycombing’) may be seen on a chest x-ray
Name 4 features of CT in Pulmonary fibrosis?
- Honeycomb appearance
- Traction bronchial dilatation (“traction bronchiectasis”)
- Thickened interlobular septae
- Reduced lung volumes
Name 4 results of the lung function tests?
- Restrictive
- Reduced FEV1/FVC - normal FEV1/FVC ratio
- Reduced lung volumes
- Reduced transfer factor - impaired gas exchange (reduced TLCO)
What is required to make a diagnosis of IPF?
high-resolution CT scanning is the investigation of choice and required to make a diagnosis of IPF
What is found on ausculatation of IPF?
bibasal fine end-inspiratory crepitations on auscultation
Name 3 clinical features of Pulmonary fibrosis?
- progressive exertional dyspnoea
- dry cough
- clubbing
What may a blood test show in 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
Management of Pulmonary fibrosis
- pulmonary rehabilitation
- Antifibronlytic theraoy
- Oxygen therapy
Name 1 antifibrolytic agent used in the treatment of pulmonary fibrosis?
Pirfenidone
What is the prognosis of someone with pulmonary fibrosis?
Average life expectancy 3-4 years
