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Progressive pulmonary fibrosis — SCE Respiratory MCQ

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HardInterstitial Lung DiseaseProgressive pulmonary fibrosisSCE Respiratory

A 62-year-old man has progressive fibrosing ILD not meeting IPF criteria. Despite antigen avoidance and immunosuppression, FVC falls by 11% predicted over 12 months and HRCT shows increasing fibrosis. What is the most appropriate management?

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Correct answer: A — Consider antifibrotic therapy for progressive pulmonary fibrosis in an ILD MDT

A relative FVC decline of at least 10% with increasing fibrosis despite appropriate management supports a progressive pulmonary-fibrosis phenotype. NICE permits nintedanib for selected chronic progressive fibrosing interstitial lung diseases. The decision belongs in an ILD multidisciplinary team, including review of the underlying inflammatory disease, adverse effects and transplant suitability.

Reference: https://www.nice.org.uk/guidance/ta747