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Systemic sclerosis-associated ILD — SCE Respiratory MCQ

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HardInterstitial lung diseaseSystemic sclerosis-associated ILDSCE Respiratory

A 49-year-old woman with systemic sclerosis has increasing exertional dyspnoea. HRCT shows bilateral basal ground-glass opacity with fine reticulation and traction bronchiectasis but little honeycombing. FVC is 59% predicted, TLCO is 37% predicted and echocardiography shows estimated pulmonary artery systolic pressure 32 mmHg. ANA is positive with anti-Scl-70 antibodies. What is the most likely diagnosis?

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Correct answer: E — Fibrotic non-specific interstitial pneumonia

The best answer is “Fibrotic non-specific interstitial pneumonia”. The clinical pattern, physiology and imaging described are most consistent with Fibrotic non-specific interstitial pneumonia. The competing diagnoses may share individual findings, but do not account for the complete combination in the stem. The remaining choices—“Usual interstitial pneumonia unrelated to systemic sclerosis”, “Pulmonary alveolar proteinosis, after specialist assessment”, “Lymphangitis carcinomatosis, with clinical reassessment”, “Acute eosinophilic pneumonia, within a respiratory pathway”—are credible in related respiratory presentations, but each addresses a different diagnostic, staging or management decision and does not fit the decisive findings and pathway position in this stem.

Reference: British Thoracic Society guideline for bronchiectasis in adults: https://thorax.bmj.com/content/74/Suppl_1/1