Progressive fibrosis in an older ex-smoker with a definite UIP pattern
A 72-year-old retired welder and ex-smoker with a 30 pack-year history presents with 18 months of progressive exertional dyspnoea and dry cough. He has fine bibasal inspiratory crackles and finger clubbing. High-resolution CT shows bilateral peripheral and basal reticulation, honeycombing and traction bronchiectasis, with no ground-glass opacity, mosaic attenuation or nodules. Pulmonary function tests show FVC 72% predicted and DLCO 42% predicted; six-minute walk distance is 340 m with oxygen saturation falling to 86%. He has no occupational asbestos or silica exposure, no birds or mould at home, and takes no culprit drugs. ANA is 1:80, rheumatoid factor is negative, and creatinine kinase is normal. He asks whether a lung biopsy is needed and what can slow progression.
1. How would you establish the diagnosis, and is a surgical lung biopsy required?
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This presentation is typical of idiopathic pulmonary fibrosis. In the correct clinical context, a high-resolution CT showing definite usual interstitial pneumonia-peripheral, basal-predominant honeycombing with traction bronchiectasis and no features suggesting an alternative diagnosis-is sufficient for a confident diagnosis without surgical biopsy. A surgical biopsy would add risk in a 72-year-old with impaired gas transfer and is unnecessary here. A multidisciplinary team review is still appropriate. Exclude connective tissue disease with a focused history and serology; a low-titre ANA alone is not diagnostic. Revisit occupational and environmental exposures, including welding fume and mould, and review drugs. Bronchoalveolar lavage is not routine with definite usual interstitial pneumonia but may help if hypersensitivity pneumonitis or another alternative is suspected. Document baseline physiology and consider a blood test for autoimmune features.
2. What management would you recommend to slow decline and improve quality of life?
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Offer an antifibrotic-pirfenidone or nintedanib-after discussing the modest but consistent slowing of FVC decline, common adverse effects and monitoring. Provide ambulatory oxygen if indicated to maintain saturation above 90% during exertion, and refer for pulmonary rehabilitation. Vaccinate against influenza, pneumococcus, COVID-19 and respiratory syncytial virus where appropriate. Treat comorbidities such as gastro-oesophageal reflux only if symptomatic, and screen for sleep-disordered breathing and pulmonary hypertension if clinically suspected. Discuss prognosis and advance care planning early. Referral for lung transplantation is reasonable to discuss, although age and comorbidity may make him ineligible; the referral decision should not be based on age alone. Avoid immunosuppression, which is harmful in idiopathic pulmonary fibrosis.
3. Four months later he develops worsening dyspnoea, fever and new bilateral ground-glass opacities, with CRP 140 mg/L. How would you manage this deterioration?
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Suspect an acute exacerbation of idiopathic pulmonary fibrosis, but first exclude infection, pulmonary embolism, heart failure and pneumothorax with cultures, viral testing, echocardiography and CT pulmonary angiography as indicated. Acute exacerbation is a diagnosis of exclusion, supported by new bilateral ground-glass or consolidation on a background of usual interstitial pneumonia and a rise in inflammatory markers. Give broad-spectrum antibiotics and empirical high-dose corticosteroid, typically methylprednisolone 500–1000 mg daily for three days followed by tapering, although evidence is weak. Provide supportive oxygen and consider non-invasive ventilation only with clear goals of care. Prognosis is poor, so discuss ceiling of treatment early with the patient and family and involve palliative care. Antifibrotics do not treat the acute episode and should generally be continued if tolerated.