Educational draft · awaiting clinical reviewThe full textbook explains uncertainty but does not replace live national or local guidance, specialist advice, or current prescribing information.
Use a disciplined clinical, exposure, radiological and physiological framework to recognise interstitial lung disease, identify emergencies and reach a defensible multidisciplinary diagnosis.
Interstitial lung diseases are diverse disorders affecting alveoli, interstitium, small airways and pulmonary vessels in different proportions. A useful first division is known association versus idiopathic disease: connective-tissue disease, exposure, medicine, radiation and occupational dust may provide the cause, while an idiopathic interstitial pneumonia is diagnosed only after credible alternatives have been addressed. The clinical aim is not merely to label an HRCT image, but to find a diagnosis that explains the person, predicts behaviour and changes management.
Work in parallel rather than serially. Define tempo and severity, assemble a lifetime exposure and medicine timeline, examine for extrapulmonary clues, measure physiology and obtain protocolled HRCT. The respiratory physician, thoracic radiologist, specialist nurse and, where needed, pathologist or rheumatologist combine these strands at multidisciplinary discussion. Bronchoscopy or biopsy is justified only if the result is likely to alter a decision and procedural risk is acceptable.
The phrase progressive pulmonary fibrosis describes behaviour across several fibrotic ILDs rather than one cause. Worsening symptoms, physiological decline and radiological progression must be interpreted together after excluding infection, cardiac disease, poor test quality and other explanations. Antifibrotic eligibility is a specialist commissioning decision; inflammatory disease may instead require exposure removal or immunomodulation.
Months or years of exertional breathlessness and dry cough, fine basal inspiratory crackles, clubbing, restrictive physiology and reduced gas transfer suggest fibrotic ILD. Functional limitation may precede resting hypoxaemia.
Subacute cough, breathlessness, feverishness, weight loss or symptoms varying with home or work exposure raise hypersensitivity pneumonitis, organising pneumonia, drug reaction, sarcoidosis or connective-tissue disease.
Raynaud phenomenon, synovitis, skin tightening, photosensitive rash, proximal weakness, uveitis, erythema nodosum, neuropathy or renal abnormalities can redirect an apparently idiopathic lung presentation.
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.
Disproportionate breathlessness, syncope, chest pain, raised jugular venous pressure, oedema or a gas-transfer reduction out of proportion to volumes warrants urgent evaluation for pulmonary vascular disease.
Haemoptysis, focal persistent opacity, unilateral effusion, pleural nodularity, unexplained weight loss or asymmetric lymphadenopathy should trigger the relevant urgent cancer pathway rather than being absorbed into an ILD label.
Oxygen does not treat fibrosis or breathlessness without hypoxaemia. Ambulatory and long-term prescriptions require formal local assessment; changing requirement is a severity signal needing review.
Confirm the specific indication and specialist eligibility. Diarrhoea, nausea, liver injury, bleeding risk, arterial events, gastrointestinal perforation risk and pregnancy precautions require counselling and monitoring; interactions and hepatic impairment can alter use.
Usual interstitial pneumonia is a radiological or pathological pattern. IPF is one clinical diagnosis associated with it, but exposure and autoimmune causes must be considered.
A job title can conceal cutting engineered stone, cleaning pigeon lofts or spraying isocyanates. Tasks, materials, ventilation and respiratory protection are more informative than occupation alone.
Combined pulmonary fibrosis and emphysema may preserve spirometric volumes while gas transfer and exertional oxygenation are severely impaired.
It can represent inflammation, infection, oedema, haemorrhage, partial alveolar filling or fine fibrosis. Distribution, accompanying signs and clinical tempo determine meaning.
Exposure information, evolving systemic features or serial imaging may revise an MDT diagnosis. A documented working diagnosis should invite appropriate re-evaluation rather than create false certainty.
Starting corticosteroids for every bilateral ground-glass opacity before adequately excluding infection, oedema and drug toxicity.
Treating a radiology label as a complete diagnosis without exposure, medicine and autoimmune review.
Using a one-off forced vital capacity to reassure despite falling TLCO, desaturation or functional decline.
Ordering broad serology without clinical interpretation, then overcalling disease from an isolated antibody result.
Delaying palliative symptom support and advance care planning until all disease-modifying options are exhausted.
Proceeding to lung biopsy when the likely result would not alter management or procedural risk is disproportionate.
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A 69-year-old has eighteen months of exertional breathlessness, dry cough, fine basal crackles and reduced gas transfer. A chest radiograph shows basal reticulation. What is the most appropriate next diagnostic approach?
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.
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