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A structured approach to interstitial lung disease

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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.

Management branches

First assessmentBuild the diagnostic matrix

Persistent breathlessness, cough, crackles or imaging suggests diffuse parenchymal lung disease.

  1. Assess physiological stability and oxygenation first; arrange same-day care for acute hypoxaemia, haemodynamic compromise, haemoptysis or rapidly progressive symptoms.
  2. 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.
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Sources and review status4 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.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom