IPF Flashcards
1
Q
ILD
A
- Lung diseases affecting the tissues and spaces surrounding the air sacs of the lungs
- IPF is the most common
2
Q
IPF
A
rare, chronic, idiopathic diseases that manifests over several years and is characterised by sccar tissue in the lungs and progressive dyspnoea
3
Q
restrictive or obstructive?
A
restrictive
4
Q
other names for it
A
- usual interstitial pneumonia
- cryptogenic fibrosing alveolitis
5
Q
risk factors
A
- smoking
- occupational/environmental exposure to metal dust, wood dust, livestock farming, birds, stone
6
Q
how does GORD predispose one to IPF
A
injury by acid aspiration
7
Q
is this an inflammatory disease?
A
no
8
Q
pathyphysiology
A
- injury results from repeated stimuli
- fibroblasts are recruited, which aggregate to form fibroblastic foci
- honeycombing
9
Q
honeycombing
A
enlarged air spaces with thick fibrotic walls lined by bronchial epithelium
10
Q
clinical features
A
- clubbing
- crackles - fine end respiratory
- cyanosis
- cough - dry
- progressive dyspnoea
- weight loss, fatigue and malaise
11
Q
what happens to the crackles as the disease advances
A
become more prominent throughout inspiration
patient becomes centrally cyanosed
12
Q
usual age of onset
A
6th decade of life
13
Q
CXR
A
- bilateral and asymmetrical changes
- reticular shadowing (opacities) which are more prominent towards the base of the lung
- fine - ground glass
- medium - honeycombing
- as the disease progresses, scarring occurs and leads to a reduction in lung volume
- shaggy heart borders
14
Q
honeycombing as a prognostic indicator
A
poor, irreversible
15
Q
imaging
A
- CXR
- HR CT
16
Q
PFT
A
show a restrictive defect
