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Idiopathic Pulmonary Fibrosis — RACP Adult Medicine MCQ

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ModerateRespiratoryIdiopathic Pulmonary FibrosisRACP Adult Medicine

A 45-year-old woman presents with worsening breathlessness over 3 years. Spirometry shows FVC 62% predicted and FEV₁ 68% predicted with FEV₁/FVC ratio 0.85. DLCO is 45% predicted. HRCT shows bilateral basal predominant honeycombing and traction bronchiectasis. What is the most likely diagnosis?

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Correct answer: CIdiopathic pulmonary fibrosis

Restrictive spirometry (normal/high FEV₁/FVC ratio, reduced FVC), severely reduced DLCO, and HRCT showing usual interstitial pneumonia pattern (basal predominant honeycombing with traction bronchiectasis) in a patient without identifiable cause is IPF. Antifibrotic therapy (nintedanib or pirfenidone) slows progression. Lung transplant assessment should be considered.

Reference: eTG Respiratory – 2024 – IPF; ATS/ERS/JRS/ALAT 2022 IPF Guidelines