Idiopathic pulmonary fibrosis Flashcards
1
Q
What is IPF?
A
progressive fibrosis of the interstitium of the lungs where no cause is identified
2
Q
Common demographic of patients with IPF
A
Male> females (2:1)
Aged 50-70
3
Q
Clinical features of IPF
A
progressive exertional dyspnoea
bibasal fine end-inspiratory crepitations
dry cough
clubbing
4
Q
Typical spirometry pattern seen in IPF
A
Restrictive
5
Q
IPF causes impaired gas exchange. What investigation can confirm this?
A
reduced transfer factor (TLCO)
6
Q
Signs of IPF on imaging
A
bilateral interstitial shadowing
(small, irregular, peripheral opacities - ‘ground-glass’ - later progressing to ‘honeycombing’)
7
Q
Imaging of choice for diagnosis of IPF
A
CT
8
Q
Management of IPF
A
- pulmonary rehab
- pirfenidone (an antifibrotic agent)
- supplementary oxygen
- lung transplant
9
Q
Prognosis/ life expectancy in IPF
A
poor prognosis
average life expectancy ~3-4 years
10
Q
A
