Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort
| Autor: | Lesley Ann Saketkoo, Virginia D. Steen, Karen A. Fagan, Paula O. Lauto, Jessica K. Gordon, Matthew R. Lammi |
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| Rok vydání: | 2017 |
| Předmět: |
030203 arthritis & rheumatology
Pulmonary and Respiratory Medicine medicine.medical_specialty business.industry Proportional hazards model Hazard ratio 030204 cardiovascular system & hematology medicine.disease Pulmonary hypertension Scleroderma Surgery 03 medical and health sciences 0302 clinical medicine Blood pressure Internal medicine medicine.artery Cohort Pulmonary artery medicine Cardiology Pulmonary wedge pressure business |
| Zdroj: | Respirology. 22:1386-1392 |
| ISSN: | 1323-7799 |
| DOI: | 10.1111/resp.13067 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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