1
Clinical Features
Examination
- Bedside clues: Oxygen, medications, mobility aids.
- Reduced bilateral chest expansion.
- Finger clubbing.
- Look for clues to the cause: irregular pulse (amiodarone usage), arthritis (deformed fingers or rheumatoid nodules, proximal myopathy, sclerodactyly, Raynaud phenomenon.
- Central/peripheral cyanosis
- Fine late-inspiratory bibasal 'Velcro' crepitations, unchanged on coughing, improve on leaning forward
- Features of right heart decompensation (JVP, peripheral edema)
PACES Pearls
- Fine bibasal end-inspiratory crepitations + clubbing + reduced expansion should make fibrotic ILD your leading bedside diagnosis.
- Look for an obvious cause and consequence.
2
Differential Diagnosis
- Pulmonary oedema
Fine bibasal crepitations can be similar, but raised JVP, peripheral oedema, an S3 and radiographic congestion favour cardiac failure. - Bronchiectasis
Crackles are usually coarse, may change after coughing, and a productive cough/sputum pot strongly favours bronchiectasis. - Resolving pneumonia
Usually gives a more focal or recent infective pattern rather than chronic symmetrical bibasal crepitations.
3
Investigations
A to E assessment for unwell patients including ABG
- Focused history
- Blood tests: FBC, U&E, LFT, CRP
- Chest X-ray: Reticular/interstitial shadowing, reduced lung volumes or alternative pathology.
- HRCT chest: honeycombing, reticulation, traction bronchiectasis, and ground-glass opacities*
- Spirometry + lung volumes + TLCO: Demonstrate a restrictive defect (FEV₁/FVC ratio is high typically ≥ 70% and quantify gas-transfer impairment.
- Resting/exertional oxygen assessment: Document desaturation and functional limitation, often with a 6-minute walk test.
- Autoimmune and exposure work-up: Target serology and occupational/environmental/drug history to the phenotype.
- ILD MDT review: Integrate clinical, radiological and, when needed, pathological information before final diagnosis and treatment.
*Honeycombing: Clustered, thick-walled cystic airspaces that signify end-stage lung fibrosis and architectural distortion.
Reticulation: A net-like or mesh pattern of fine lines caused by thickened interlobular and intralobular interstitium.
Traction Bronchiectasis: Airways pulled open and distorted by surrounding scarred and shrinking lung tissue.
Ground-Glass Opacities (GGO): Hazy, whitish areas of increased lung density where airspaces are partially filled or interstitium is mildly inflamed, allowing blood vessels to remain visible
4
Management
- Cause-specific treatment: Remove an offending drug/antigen and treat connective-tissue or inflammatory disease with specialist advice.
- Fibrotic ILD/IPF: Consider antifibrotic therapy through a specialist ILD service
- Pulmonary rehabilitation: Offer exercise and education-based rehabilitation when functionally limited.
- Oxygen: Assess for ambulatory or long-term oxygen when hypoxaemia meets appropriate criteria.
- Supportive care: Vaccination, smoking cessation, symptom control, nutrition and early discussion of palliative/supportive needs in progressive disease.
- Transplant referral: Consider early in suitable patients with progressive advanced disease rather than waiting for end-stage deterioration.
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