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Scleroderma lung disease — SCE Rheumatology MCQ

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ModerateConnective tissue diseasesScleroderma lung diseaseSCE Rheumatology

A 50-year-old woman with systemic sclerosis reports progressively worsening exertional dyspnoea. Fine bibasal late-inspiratory crackles are present. Over 12 months, her FVC has fallen by 12% from baseline and her DLCO is reduced. Transthoracic echocardiography performed 3 months ago showed no evidence of pulmonary hypertension. Which investigation should now be performed to identify and characterise the most likely pulmonary complication?

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Correct answer: B — High-resolution CT of the chest

The correct answer is **B, high-resolution CT of the chest**. Progressive dyspnoea, bibasal crackles, a clinically important decline in FVC and reduced DLCO strongly suggest systemic sclerosis-associated interstitial lung disease (SSc-ILD). HRCT is the primary test to confirm, characterise and determine the extent of parenchymal ILD; repeat imaging is appropriate when respiratory symptoms or pulmonary function deteriorate. Standard chest radiography lacks sensitivity for early or limited ILD. Pulmonary hypertension may reduce DLCO, but the restrictive physiological decline and crackles, together with a recent reassuring echocardiogram, make this less likely as the principal explanation. CT pulmonary angiography is for suspected pulmonary embolism, and bronchoalveolar lavage is not routinely required to diagnose SSc-ILD.

Reference: Antoniou KM et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease, 2026; systemic sclerosis diagnostic and monitoring algorithms. https://pubmed.ncbi.nlm.nih.gov/40907995/