A 42-year-old woman with diffuse cutaneous systemic sclerosis develops acute onset dyspnea, pulmonary crackles, and an oxygen saturation of 88%. CT chest shows ground-glass opacities and reticular pattern predominantly in lower lobes. Pulmonary function tests show FVC 52%, DLCO 44%. What is the first-line treatment for this pulmonary complication?

• A High-dose corticosteroids alone
• B Bosentan (endothelin receptor antagonist)
• C Cyclophosphamide IV as monotherapy
• D Mycophenolate mofetil or nintedanib ✓

Explanation

SSc-associated ILD is the leading cause of mortality in diffuse SSc. Mycophenolate mofetil is first-line disease-modifying therapy (SLS II trial showed equivalence to cyclophosphamide with better tolerability). Nintedanib (a tyrosine kinase inhibitor) is approved for SSc-ILD based on the SENSCIS trial showing reduced FVC decline and can be used alone or with MMF. High-dose steroids increase risk of scleroderma renal crisis and are generally avoided. Bosentan targets PAH, not ILD.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.