Interstital lung diseases - IDIOPATHIC PULMONARY FIBROSIS Flashcards
What are interstitial lung diseases?
any disease affecting the interstitium of the lung
what is idiopathic pulmonary fibrosis?
= a type of lung disease that results in fibrosis (scarring) of the lungs for an UNKNOWN REASON
what is the cause of idiopathic pulmonary fibrosis?
the cause is UNKNOWN
although the exact cause of idiopathic pulmonary fibrosis is not known, what are some suggestions and some secondary causes?
Some suggestions;
- imbalance of fibrotic repair system
- related to gastric reflux
Secondary causes;
- smoking
- rheumatoid
- systemic lupus erythematosous
- systemic sclerosis
- asbestos
- drugs (e.g. methotrexate)
what is the histological finding in idiopathic pulmonary fibrosis?
Usual interstice pneumonia (UIP)
what are the key pathological features of idiopathic pulmonary fibrosis?
- heterogenous appearance
- ares of normal with areas of FIBROSIS (scarring)
- honeycombing in sub-pleural arreas
- fibro-blastic focci
- inflammation is minimal
what effect does IPF have on gas exchange?
IPF leads to impaired gas exchange which eventually leads to progressive respiratory failure
what are the clinical features of IPF?
- onset of progressive dyspnoea
- breathlessness
- may be accompanied by a cough
- cough may have or may not have sputum production
- finger clubbing
- bilateral fine inspiratory crackles
- progressive respiratory failure
what age of patients are at great risk of getting IPF?
- patients in their sixties
- it is very rare below the age of 50
what 5 investigations could be done to reach the diagnosis of IPF?
1) respiratory function tests
2) blood tests
3) Chest X-ray
4) High resolution CT scan
5) bronchoalveolar lavage
what sort of pattern of disease would the respiratory function tests show?
would show RESTRICTIVE PATTERNS of disease.
- reduced FEV1 & FVC
- reduced lung volumes
- reduced gas exchange
- normal or raised ratio
what would the chest X-ray show?
- bilateral infiltrates
- small volume lungs
- increased reticular shadowing at the bases
What 4 things would a CT scan show?
1) basal disturbances
2) sub pleural reticulation
3) traction bronchiectasis
4) honeycombing
why are basal disturbances seen on a?
abnormalities are more pronounced at the absence
Where is reticulation most evident on a CT scan?
in the lung peripheries
