Synopsis
Recognise the characteristic fibrotic presentation, establish idiopathic pulmonary fibrosis through multidisciplinary exclusion of alternatives and deliver safe antifibrotic and supportive care.
- IPF is a chronic progressive fibrosing interstitial pneumonia of unknown cause, usually occurring in older adults and confined to the lungs.
- Typical clues are insidious exertional breathlessness, dry cough, fine basal inspiratory crackles, clubbing and a restrictive or gas-transfer defect.
- The diagnosis requires exclusion of domestic, occupational, medicine and connective-tissue causes; 'idiopathic' is not established by HRCT alone.
Key red flags
New severe dyspnoea over usually less than one month with new bilateral ground-glass opacity or consolidation superimposed on UIP is life-threatening after oedema and other causes are addressed.
Investigation priorities
Determine whether the fibrosis has a UIP probability pattern.
Management branches
Chronic fibrotic ILD is suspected in an appropriate adult.
- Document clinical tempo, smoking, full medicine and exposure histories, and targeted connective-tissue findings; obtain complete physiology and protocolled HRCT.
- Present clinical and radiological evidence at an ILD multidisciplinary meeting to judge UIP pattern, credible causes and diagnostic confidence.
Key medicines
NintedanibSpecialist initiation is normally 150 mg orally twice daily approximately twelve hours apart with food; reduce to 100 mg twice daily, interrupt or discontinue for intolerance as directed by the current SmPC.
PirfenidoneSpecialist-supervised titration over fourteen days: 267 mg three times daily for days 1–7, 534 mg three times daily for days 8–14, then 801 mg three times daily with food, subject to current product instructions.