Idiopathic pulmonary fibrosis Flashcards
1
Q
What is idiopathic pulmonary fibrosis
A
type of ILD
progressive fibrosis of the interstitial
2
Q
What age is it typically seen in?
A
50-70
3
Q
What are the sx?
A
SOB - progressive exertional
Cough - non productive
WL, fatigue, malaise
4
Q
what can be found on examination
A
end inspiratory basilar crackles
clubbing
5
Q
What are the investigations? What is essential for diagnosis?
A
- HRCT - basilar, sub pleural areas of ↑ reticulation, honeycombing + traction bronchiectasis
- CXR - small volume lungs w ↑ reticular shadowing at bases
- Spirometry - reduced FVC + TLC, increased FEV1/FVC ratio
- Bloods - ABG (low o2, ↑CRP, Ig)
- ANA + RF - exclude autoimmune rheumatic disease
6
Q
What is the treatment?
A
- Monitor disease functioning w LFTs
- Supportive care: O2, pulmonary rehabilitation
- Pirfenidone - antifibrotic agent
- Lung transplant
7
Q
What drug must not be given?
A
high dose steroids
