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.
A structured approach to interstitial lung disease
Essential points for quick revision.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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Escalate
Interstitial lung disease is usually assessed electively, but new severe hypoxaemia, rapidly increasing oxygen need, respiratory exhaustion, shock, haemoptysis or an acute diffuse infiltrative process requires same-day hospital assessment. Stabilise airway, breathing and circulation, give oxygen to an appropriate target, investigate infection, oedema, embolism and pneumothorax, and involve respiratory and critical-care teams early rather than assuming an 'ILD flare'.
Synopsis
Use a disciplined clinical, exposure, radiological and physiological framework to recognise interstitial lung disease, identify emergencies and reach a defensible multidisciplinary diagnosis.
ILD is an umbrella description, not a final diagnosis: classify the clinical context, HRCT pattern and likely cause before selecting treatment.
Take an exposure history that reaches beyond occupation to birds, mould, hot tubs, humidifiers, hobbies, dusts, vaping, radiotherapy and every prescribed or non-prescribed medicine.
Search actively for connective-tissue disease through Raynaud phenomenon, inflammatory joints, rashes, proximal weakness, sicca symptoms, dysphagia and mechanic's hands.
Key red flags
Acute hypoxaemic deterioration
A rapid fall over days with new bilateral opacities, marked desaturation or increased work of breathing is an emergency. Consider infection, embolism, oedema, pneumothorax, haemorrhage, drug toxicity and acute exacerbation.
Investigation priorities
01
Structured history and examinationFirst step
Identify cause, tempo, comorbidity and immediate severity.
Assess physiological stability and oxygenation first; arrange same-day care for acute hypoxaemia, haemodynamic compromise, haemoptysis or rapidly progressive symptoms.
Construct symptom, medicine and lifetime exposure timelines, examine for connective-tissue and sarcoid features, then obtain chest imaging, full lung function and targeted blood tests.
Key medicines
Supplemental oxygenIn acute illness titrate to 94–98% for most adults or 88–92% when hypercapnic respiratory failure risk is present, pending blood gases and any individual target.
Nintedanib for eligible progressive fibrosing ILDSpecialist initiation is usually 150 mg orally twice daily about twelve hours apart with food; 100 mg twice daily or interruption may be required for intolerance, following the current SmPC.
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.
BTS interstitial lung disease guidelineArchived 2008 document used only for enduring diagnostic principles; current specialist and medicine guidance takes precedence.