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This is a clinician-written, evidence-based summary aligned to the 2026 MLA Content Map. It is intended for medical students and junior doctors preparing for the UKMLA. Always cross-reference with NICE guidance, local protocols, and clinical judgement.
Key points
- Progressive fibrosing ILD of unknown cause — median survival 3–5 years from diagnosis
- Typical patient: male, >60, smoker or ex-smoker, progressive breathlessness + dry cough
- Examination: bibasal fine inspiratory crackles ("velcro crackles") + finger clubbing
- HRCT: usual interstitial pneumonia (UIP) pattern — honeycombing, traction bronchiectasis, predominantly basal and peripheral
- Treatment: antifibrotic therapy — pirfenidone or nintedanib (NICE TA504/TA379) slows progression but does NOT cure
Overview
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. It is the commonest and most lethal of the idiopathic interstitial pneumonias. The histological and radiological pattern is usual interstitial pneumonia (UIP), characterised by spatial and temporal heterogeneity of fibrosis with honeycombing. The condition is limited to the lungs and predominantly affects the lower lobes. IPF must be distinguished from other causes of pulmonary fibrosis including connective tissue disease-associated ILD, drug-induced fibrosis, hypersensitivity pneumonitis, and sarcoidosis.
Epidemiology
IPF has an estimated UK incidence of approximately 7–10 per 100,000, increasing with age. It predominantly affects men over 60, with a strong association with current or previous smoking. Other risk factors include occupational exposures (metal and wood dust, farming), gastro-oesophageal reflux, and genetic predisposition (mutations in telomerase genes). Median survival from diagnosis is approximately 3–5 years, though the clinical course is variable — some patients remain stable while others experience rapid decline or acute exacerbations.
Clinical Features
Symptoms
Progressive exertional breathlessness (insidious onset over months to years)
Persistent dry cough (non-productive)
Reduced exercise tolerance
Fatigue and weight loss in advanced disease
Signs
Bibasal fine end-inspiratory crackles ("velcro crackles") on auscultation — the hallmark
Finger clubbing (present in ~50%)
Cyanosis in advanced disease
Signs of cor pulmonale (peripheral oedema, raised JVP) in end-stage disease
Investigations
First-line
High-resolution CT (HRCT) thoraxDefinitive non-invasive diagnosis — UIP pattern: honeycombing, traction bronchiectasis, basal and peripheral predominance, minimal ground-glass
SpirometryRestrictive pattern: reduced FVC and TLC, normal or raised FEV1/FVC ratio
TLCO (gas transfer)Reduced — often the earliest abnormality and best marker of disease severity
Second-line
Autoimmune screen (ANA, RF, anti-CCP, myositis antibodies)Exclude connective tissue disease-associated ILD
Serum precipitins (avian, mould)Exclude hypersensitivity pneumonitis
EchocardiogramAssess for pulmonary hypertension
Specialist
Multidisciplinary team (MDT) discussionDiagnosis should be confirmed at a specialist ILD MDT (radiologist, pathologist, respiratory physician)
Surgical lung biopsyOnly if HRCT is indeterminate and diagnosis would change management — carries significant risk in IPF patients
1
Antifibrotic therapy
- Pirfenidone (NICE TA504) or nintedanib (NICE TA379) — both slow FVC decline but do NOT reverse fibrosis
- Criteria: FVC 50–80% predicted at specialist ILD centre
- Monitor LFTs regularly (hepatotoxicity risk with both)
- Common side effects: pirfenidone (photosensitivity, GI upset, rash); nintedanib (diarrhoea, hepatotoxicity)
2
Supportive care
- Pulmonary rehabilitation — improves exercise capacity and quality of life
- Supplemental oxygen if resting PaO2 <7.3 kPa or desaturation on exertion
- Manage comorbidities: GORD treatment (may slow progression), cough management (low-dose opioids)
- Psychological support — depression and anxiety are common
3
Lung transplant and palliative care
- Refer for lung transplant assessment if age <65 and no major comorbidities
- Advance care planning — discuss prognosis and preferences early
- Palliative care involvement for symptom management in progressive disease
4
Acute exacerbation of IPF
- Acute worsening not explained by infection, PE, or heart failure — carries >50% in-hospital mortality
- No proven treatment — high-dose steroids are often given empirically but evidence is weak
- Exclude and treat reversible causes
Complications
- Progressive respiratory failure: Inexorable decline in lung function
- Pulmonary hypertension: Develops in advanced disease, worsens prognosis
- Lung cancer: Increased risk (5–10 times) — particularly in areas of honeycombing
- Acute exacerbation: Rapid deterioration with bilateral ground-glass opacities — mortality >50%
- Pneumothorax: From subpleural honeycombing
UKMLA Exam Tips
- 1Bibasal fine "velcro" crackles + finger clubbing + restrictive spirometry + UIP on HRCT = IPF
- 2UIP pattern on HRCT: honeycombing + traction bronchiectasis + basal/peripheral predominance = diagnostic in the right clinical context
- 3Pirfenidone and nintedanib SLOW progression but do NOT cure — neither reverses established fibrosis
- 4TLCO is the most sensitive early marker and the best predictor of prognosis
- 5Do NOT prescribe steroids or immunosuppressants for IPF — the PANTHER trial showed harm (increased mortality with triple therapy)
- 6IPF is associated with increased lung cancer risk
- 7Median survival 3–5 years — worse than many cancers; early palliative care is important
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