Bronchiolitis obliterans syndrome (BOS) is the most common form of chronic lung allograft dysfunction after lung transplant, marked by progressive small airway disease with limited treatment options. This case report describes a 55-year-old woman with primary ciliary dyskinesia, post-bilateral lung transplant, who developed severe (stage 3) bronchiolitis obliterans syndrome and went into respiratory failure requiring mechanical ventilation. After starting sotatercept off-label — a drug approved for pulmonary arterial hypertension that targets the activin signaling pathway — she showed rapid clinical and imaging improvement, and was temporarily able to come off the ventilator, though she ultimately died from progressive respiratory failure.
The authors note this suggests activin pathway modulation may warrant further study as a potential approach for advanced BOS, though this is a single case, not a clinical trial.
Read more at this link on the JHLT Open
Citation
Poor H, Zhang J, Hannah-Clark S …Sotatercept treatment in a patient with end-stage bronchiolitis obliterans syndrome after lung transplantationJHLT Open, 2026; 13 DOI: 10.1016/j.jhlto.2026.100585