Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort.

BACKGROUND AND OBJECTIVE: Systemic sclerosis (SSc) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH). When associated with elevated pulmonary artery wedge pressure (PAWP), pulmonary artery pressure (PAP) is either in-proportion (post-capillary PH) or higher than expected (combined PH) relative to the increased PAWP. METHODS: Patients from the PHAROS registry (a prospective observational cohort of SSc-PH patients) who had mean PAP ≥ 25 and PAWP > 15 on right heart catheterization were stratified based on diastolic pressure gradient (DPG). Kaplan-Meier analysis was performed to compare survival and PH-related hospitalization. Baseline factors were compared between patients dying and those who survived using Cox regression analysis. RESULTS: A total of 59 patients were included, of whom 21 (36%) patients were classified as combined PH and 38 (64%) had post-capillary PH. No baseline characteristics were significantly different between the two groups. There were no differences in survival or PH-related hospitalization between the groups. The only baseline factor independently associated with death was lower 6-min walk distance (6MWD) (hazard ratio (HR): 1.33 per 25 m decrease, 95% CI: 1.11-1.59, P = 0.002). PH-specific medications were started during follow-up in significantly more patients in the combined PH group compared with the post-capillary group (86% vs 50%, P = 0.01). CONCLUSION: Outcomes were similar between SSc patients with post-capillary PH and combined pre- and post-capillary PH. 6MWD at baseline can predict risk for death in SSc patients with PH and an elevated PAWP. More patients with combined PH were started on PH-specific medications, and the clinical benefit of treating this subgroup specifically in SSc patients needs further exploration.

A multicenter, retrospective study of patients with pulmonary arterial hypertension transitioned from parenteral prostacyclin therapy to inhaled iloprost.

Pulmonary arterial hypertension (PAH) is characterized by progressive increases in pulmonary vascular resistance, leading to right heart failure and death. Guidelines recommend customization of treatment, necessitating the development of effective strategies for transitioning patients among treatments. In this study, we characterized our experience with patient transitions from parenteral prostacyclin to inhaled iloprost. We retrospectively assessed records from 11 centers of 37 consecutive patients with PAH aged ≥ 18 years who were treated with intravenous (IV) or subcutaneous (SC) prostacyclin analogues and transitioned to inhaled iloprost. The transition period began on the first day of inhaled iloprost with the intent of discontinuing parenteral prostacyclin and ended on the first day on inhaled iloprost free of parenteral prostacyclin. Persistence was defined as the absence of (1) parenteral prostacyclin while remaining on inhaled iloprost during post-transition Days 1-90 and (2) no reinitiation of parenteral prostacyclin during post-transition Days 90-365. All patients were clinically stable before transitioning to inhaled iloprost. The mean age was 46.5 years, 70.3% were female, 51.4% had idiopathic PAH, and 43.0% were in New York Heart Association Functional Class III. Among patients with an overlapping transition, the mean transition period was 10.5 days. A transition dosing algorithm was used in 10 patients (27.0%). At one year, 78.4% of the patients remained persistent on inhaled iloprost and 81.1% were free of clinical worsening. In selected patients on background oral PAH therapy, transitioning from parenteral prostacyclin to inhaled iloprost appears safe and feasible and is associated with long-term success. Further study is needed to define the optimal patient selection criteria and transition algorithm.