Survival Prediction in All-cause Systemic Sclerosis-associated Pulmonary Hypertension

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Abstract Rationale Pulmonary hypertension (PH) is a leading cause of mortality in patients with systemic sclerosis (SSc). SSc patients are at risk for the development of multiple phenotypes of PH, including World Health Organization (WHO) Group 1 pulmonary arterial hypertension (SSc-PAH), WHO Group 2 secondary to left heart disease (SSc-PH-LHD), and WHO Group 3 secondary to interstitial lung disease (SSc-PH-ILD). Multiple, overlapping phenotypes of PH may co-exist within the same SSc patient. We therefore sought to identify survival predictors in an aggregated incident SSc-PH population. Methods We utilized the PHAROS registry to include patients with an incident diagnosis of PH classified as either WHO Group 1, 2, or 3. We compared baseline demographic, clinical, and hemodynamic variables between the PH groups using one-way ANOVA. Survival was compared between the PH groups using Kaplan-Meier analysis. Finally, predictors of overall survival for the aggregated SSc-PH group were identified using a multivariable Cox regression model employing a LASSO variable selection method. Results We included a total of 323 patients: 209 PAH, 52 PH-LHD and 62 PH-ILD. Mean age was 58.0±10.6 years, with PH-LHD the oldest (65.3±11.8 years). There were no differences in 6-minute walk distance or WHO functional class between the PH groups. There were no significant survival differences between PH groups 1-3 (log-rank=0.95). Significant predictors of mortality were found to be Age (HR 1.03; 95% CI 1.00-1.05, for every 1-year increase), Sex (HR 0.32; 95% CI 0.18-0.53, for females), Race (HR 1.81; 95% CI 1.02-3.20, for non-Hispanic whites), 6MWD (HR 0.96; 95% CI 0.94-0.99, for each additional 10 meters walked), and mPAP (HR 1.01; 95% CI 1.01-1.06 for each 1 mmHg). A cut-point for DLCO was created due to the violation of proportional hazards assumption, with DLCO significantly associated with survival only during first 4.5 years of PH (HR 0.94; 95% CI 0.92 – 0.96 for each 1% predicted). Conclusions We did not identify any survival differences between SSc-PH groups, underscoring the potential impact of overlapping phenotypes within the same patient. Demographics and 6MWD remain strongly associated with outcomes in SSc-PH, while mPAP was a better predictor than any other invasive hemodynamic parameter including PVR. Table 1.

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