Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.
Idiopathic pulmonary fibrosis
Respiratory · UK scope · Sources checked 27 Aug 2026 · Clinical review pending
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Escalate
A person with IPF who develops marked new breathlessness, hypoxaemia, chest pain, fever, haemoptysis or rapidly increasing oxygen requirement needs urgent hospital assessment. Manage immediate respiratory failure and seek infection, pulmonary embolism, cardiac failure, pneumothorax and other causes before labelling an acute exacerbation; obtain early respiratory and critical-care advice consistent with documented preferences and treatment ceilings.
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.
A usual interstitial pneumonia pattern favours subpleural basal reticulation, traction bronchiectasis and honeycombing, without features suggesting an alternative diagnosis.
When clinical context and HRCT are sufficiently characteristic, surgical lung biopsy is unnecessary and may expose the patient to serious harm.
Antifibrotics slow physiological decline but do not reverse established fibrosis; benefits, adverse effects and stopping rules should be discussed honestly.
Nintedanib and pirfenidone eligibility follows current NICE technology appraisals, specialist prescribing and local commissioning arrangements.
Pulmonary rehabilitation, smoking cessation, vaccination, oxygen assessment and symptom-focused care should begin alongside disease-modifying discussion.
Serial decline, exertional desaturation and pulmonary hypertension matter prognostically even if resting saturation or spirometric volumes appear acceptable.
Lung transplantation should be considered early enough for assessment in potentially suitable patients, rather than after irreversible deconditioning or crisis.
Key red flags
Acute exacerbation syndrome
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.
Pulmonary vascular complication
Breathlessness, syncope, oedema or oxygen need disproportionate to lung volumes raises pulmonary hypertension, pulmonary embolism or cardiac disease and warrants prompt specialist evaluation.
Investigation priorities
01
High-resolution computed tomographyFirst step
Determine whether the fibrosis has a UIP probability pattern.
Management branches
DiagnosisConfirm IPF without unnecessary biopsy
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.
If confidence remains insufficient and the answer would alter care, consider lavage or tissue only after individual procedural and exacerbation risk is discussed.
Communicate the working diagnosis, uncertainty, likely course and contact route in plain language, with a plan to revisit unexpected features.
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.Check liver function before and during treatment. Diarrhoea and nausea are common; dehydration, bleeding, arterial thromboembolism and gastrointestinal perforation risks require context. Avoid in pregnancy and review anticoagulants, hepatic impairment and interacting medicines.
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.Obtain liver tests before treatment, monthly for six months and then every three months unless more frequent review is indicated. Counsel on nausea, anorexia, photosensitivity, sun protection, smoking-related reduced exposure and important CYP1A2 interactions.
Sources and review status5 sources · checked 27 Aug 2026 · clinical review pending+
Sources
Sources and review status
National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.