Dataset Information

Long-Term Outcomes in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS).

ABSTRACT:

Background

Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH.

Methods

Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of patients with SSc at high risk for or with incident pulmonary hypertension from right heart catheterization. Incident World Health Organization group I PAH patients were analyzed. Kaplan-Meier survival curves were generated for the overall cohort and those who died of PAH. Multivariate Cox regression models identified predictors of mortality.

Results

Survival in 160 patients with incident SSc-PAH at 1, 3, 5, and 8 years was 95%, 75%, 63%, and 49%, respectively. PAH accounted for 52% of all deaths. When restricted to deaths from PAH, respective survival rates were 97%, 83%, 76%, and 76%, with 93% of PAH-related deaths occurring within 4 years of diagnosis. Men (hazard ratio [HR], 3.11; 95% CI, 1.38-6.98), diffuse disease (HR, 2.12; 95% CI, 1.13-3.93), systolic pulmonary artery pressure (PAP) on ECG (HR, 1.06 95% CI, 1.01-1.11), mean PAP on right heart catheterization (HR, 1.03; 95% CI, 1.001-1.07), 6-min walk distance (HR, 0.92; 95% CI, 0.86-0.99), and diffusing capacity for carbon monoxide (HR, 0.65; 95% CI, 0.46-0.92) significantly affected survival on multivariate analysis.

Conclusions

Overall survival in PHAROS was higher than other SSc-PAH cohorts. PAH accounted for more than one-half of deaths and primarily within the first few years after PAH diagnosis. Optimization of treatment for those at greatest risk of early PAH-related death is crucial.

SUBMITTER: Kolstad KD

PROVIDER: S-EPMC6207791 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature

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Publications

Long-Term Outcomes in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS).

Kolstad Kathleen D KD Li Shufeng S Steen Virginia V Chung Lorinda L

Chest 20180516 4

<h4>Background</h4>Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH.<h4>Methods</h4>Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of patients with SSc at high risk for or with incident pulmonary hypertension from right heart catheterization. Incident World Health Organization group I PAH patients w ...[more]

PMID: 29777655

Similar Datasets

Project description:ObjectiveTo compare time to clinical worsening (TTCW) based on initial oral therapy for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc)-related PAH.MethodsUsing data from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) registry (a multicenter prospective observational study enrolling SSc patients with incident pulmonary hypertension), we selected patients with group 1 PAH (World Health Organization Clinical Classification system) who received initial therapy (for 6 months) with an endothelin receptor antagonist (ERA), a phosphodiesterase 5 (PDE5) inhibitor, or a combination of these 2 agents (ERA/PDE5 inhibitor). The main outcome was TTCW, defined as the first occurrence of death, PAH-related hospitalization, lung transplantation, initiation of parenteral prostacyclin treatment, or worsening symptoms.ResultsNinety-eight patients (24 in the ERA group, 59 in the PDE5 inhibitor group, and 15 in the ERA/PDE5 inhibitor group) were included. No significant differences in the baseline characteristics of the patients were observed. TTCW was significantly worse in patients in the ERA group compared with those in the PDE5 inhibitor group or the ERA/PDE5 inhibitor group. Ten patients (41.6%) in the ERA group died during the 3-year observation period, compared with 4 patients (6.8%) in the PDE5 inhibitor group and 1 patient (6.7%) in the ERA/PDE5 inhibitor group. Baseline factors that were independently associated with a shorter TTCW were initial treatment with an ERA (hazard ratio [HR] 2.63 [P = 0.009]), lower diffusing capacity for carbon monoxide (HR 0.69 per 10% of predicted change [P = 0.04]), and higher pulmonary vascular resistance (HR 1.10 per Wood unit change [P = 0.007]).ConclusionCompared with initial treatment with a PDE5 inhibitor or combination therapy with an ERA and a PDE5 inhibitor, initial therapy with an ERA in patients with SSc-related PAH was associated with significantly worse TTCW, even after adjustment for commonly accepted prognostic factors. Further study into the optimal initial oral therapy for patients with SSc-related PAH is needed.

Dataset Information

Long-Term Outcomes in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS).

Background

Methods

Results

Conclusions

Publications

Long-Term Outcomes in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS).

Similar Datasets

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