Improved Survival for Patients with Systemic Sclerosis–associated Pulmonary Arterial Hypertension: The Johns Hopkins Registry

The article describes a study of 504 patients with systemic sclerosis-associated pulmonary hypertension (SSc-PH) and compares their characteristics and survival between two time periods (1999-2010 and 2010-2021). The study found that patients with group 1 disease diagnosed in the more recent period had better clinical and hemodynamic characteristics, were more likely to receive combination therapy, and had a nearly 4-year increase in median transplant-free survival compared to those in the earlier period. No differences were observed in baseline characteristics or survival for patients with group 2 or 3 disease. The study concludes that transplant-free survival has improved significantly for those with group 1 disease in the last decade due to earlier detection and better management, but those with group 2 or 3 disease continue to have a poor prognosis.