Idiopathic pulmonary fibrosis
Description
- Chronic, progressive fibrosing interstitial pneumonia of unknown cause, limited to the lungs
- Histological and radiological pattern = UIP
- Diagnosis of exclusion - requires no identifiable cause after full CTD, exposure and drug assessment
The UIP pattern
- Spatial heterogeneity - normal lung adjacent to fibrosis
- Temporal heterogeneity - fibroblastic foci (young) next to dense collagen (old)
- Subpleural, basal, peripheral predominance; honeycombing
- UIP pattern is NOT specific to IPF - also chronic hypersensitivity pneumonitis, CTD-ILD (esp. RA), asbestosis, drug toxicity, familial fibrosis
Progressive pulmonary fibrosis (PPF)
- A behaviour, not a diagnosis: any non-IPF fibrosing ILD meeting 2 of 3 within 12 months
- Worsening symptoms
- dec FVC >=5% absolute or dec DLCO >=10%
- Radiological progression
- Matters because PPF is treated like IPF - antifibrotics, not immunosuppression
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