Sotatercept as a Therapeutic Option for Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension With Features of PVOD/PCH

A 53-year-old woman with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) presented with severe exertional desaturation, markedly reduced diffusing capacity (%DLCO 30%), and computed tomography findings demonstrating features of pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH). Despite triple oral therapy, pulmonary hypertension progressively worsened, with mean pulmonary arterial pressure increasing from 34 to 51 mmHg. Sotatercept was initiated because of concerns about the risk of pulmonary edema with parenteral prostacyclin therapy. Despite intermittent treatment interruption due to hemoglobin elevation, improvements were observed in World Health Organization functional class (III-II), 6-min walk distance (217-301 m), brain natriuretic peptide (206.4-52.8 pg/mL), and mean pulmonary arterial pressure (51-45 mmHg), without pulmonary edema. This case suggests a potential benefit of sotatercept in selected patients with SSc-PAH with features of PVOD/PCH.