A 50-year-old woman with a 5-year history of limited cutaneous systemic sclerosis (CREST syndrome) presents with fatigue and dyspnea. She has telangiectasias, calcinosis, Raynaud phenomenon, and anti-centromere antibodies. Right heart catheterization shows mean pulmonary artery pressure of 44 mmHg, pulmonary capillary wedge pressure of 9 mmHg, and pulmonary vascular resistance of 6 Wood units. Which is the most appropriate targeted therapy?

• A Endothelin receptor antagonist (bosentan) ✓
• B High-dose corticosteroids
• C Cyclophosphamide pulse therapy
• D Mycophenolate mofetil

Explanation

This patient has Group 1 pulmonary arterial hypertension (PAH) complicating limited SSc, confirmed by RHC (mPAP >20 mmHg, PCWP ≤15, elevated PVR). Endothelin receptor antagonists (bosentan, ambrisentan), PDE5 inhibitors, and prostacyclin analogs are first-line targeted PAH therapies. Cyclophosphamide and mycophenolate are used for scleroderma-associated ILD, not isolated PAH. Corticosteroids do not treat PAH.

Reference: Kelley and Firestein's Textbook of Rheumatology, 11th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.