Abstract
Sarcoidosis is often complicated by the development of pulmonary hypertension (PH). Sotatercept, a ligand trap for selected transforming growth factor β superfamily members, is a novel, highly effective therapy approved for the treatment of pulmonary arterial hypertension. We conducted a retrospective study of patients with biopsy-proven sarcoidosis who received sotatercept for the treatment of sarcoidosis-associated pulmonary hypertension (PH) to assess the effect of sotatercept on lung function. Nine patients were included in the study. Median age was 64 years (IQR 56 to 71), seven patients (78%) were female. All patients were receiving chronic background immunosuppression, and eight patients (89%) were receiving background pulmonary vasodilators. Pulmonary function testing following initiation of sotatercept (median 4.4 months, IQR 2.9 to 8.0 months) demonstrated improvement in lung function in all patients: FEV1 increased 150 mL (13%, IQR 65 to 340 mL), FVC increased 170 mL (11%, IQR 65 to 285 mL), DLCO increased 0.95 mL/min/mmHg (14%, IQR 0.30 to 2.59 mL/min/mmHg), and FEF25-75% increased 0.18 L/s (55%, IQR -0.04 to 0.75 L/s). One patient had near complete radiographic resolution of pulmonary infiltrates. Seven patients (78%) had stable or decreasing background immunosuppression. In patients with sarcoidosis-associated PH, sotatercept was associated with improvement in lung function, and in particular, markers of small airways disease.