Comparison of Four Risk Stratification Models for Prediction of Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in the EUSTAR Cohort

Poster Presenter: Hilde Jenssen Bjørkekjær, MD, Rheumatology Fellow and PhD Candidate, Department of Rheumatology, Hospital of Southern Norway, and University of Oslo, Institute of Clinical Medicine, Norway

Poster Title: Comparison of Four Risk Stratification Models for Prediction of Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in the EUSTAR Cohort

Poster Session A: Sunday, Nov. 12

What is your poster about?
“In our poster, we compared the performance of risk stratification tools to predict mortality in incident systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) patients from the EUSTAR cohort. We found that all the tools had a higher predictive ability compared to the established model for baseline risk assessment, the European Society of Cardiology (ESC)/European Respiratory Society (ERS) model in three strata. COMPERA 2.0 and REVEAL Lite 2 had comparable predictive ability, however, COMPERA 2.0 segregated the intermediate-risk group into two groups with significantly different long-term survival. Adding SSc-specific characteristics improved the predictive ability slightly, and diffusing capacity of the lungs for carbon monoxide (DLCO) was a predictor of mortality independent of risk stratification. Based on these results, we suggest using COMPERA 2.0 at the time of SSc-PAH diagnosis to identify patients at intermediate-high risk in addition to those at high risk where careful management is warranted. Additionally, we propose to take DLCO into account in the overall assessment.

“We also have an oral abstract presentation entitled ‘Treatment Regimens and Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in Light of the 2022 ESC/ERS Guidelines.’ Here we assessed treatment regimens according to hemodynamic thresholds (lower threshold: mean pulmonary arterial pressure (mPAP) 21-24 mmHg or pulmonary vascular resistance (PVR) 2-3 Wood units (WU) versus higher threshold: mPAP ≥25 mmHg and PVR ≥3 WU) and risk stratification (using COMPERA 2.0) and their association with mortality. We found that upfront combination therapy was used more frequently in patients with higher versus lower mPAP and PVR thresholds and in patients in the high-risk group versus lower-risk groups; however, still 40% did not receive any treatment upfront. We also found that upfront treatment is associated with improved survival, independent of higher mPAP and PVR thresholds and intermediate-high and high-risk groups. Based on these results, we suggest considering more aggressive SSc-PAH treatment.”

“Risk stratification in PAH is essential to optimize management. Previous research has demonstrated that a more aggressive treatment strategy is beneficial for SSc-PAH patients; therefore, it is crucial to identify patients at high risk of mortality.”

— Hilde Jenssen Bjørkekjær, MD

Why did you decide to investigate this topic?
“Risk stratification in PAH is essential to optimize management. Previous research has demonstrated that a more aggressive treatment strategy is beneficial for SSc-PAH patients; therefore, it is crucial to identify patients at high risk of mortality. However, risk stratification tools have been based predominantly on data from patients with idiopathic PAH (IPAH), while SSc-PAH has a worse prognosis than IPAH. Therefore, we aimed to compare the performance of risk stratification tools in a cohort of SSc-PAH patients, to assess the effect of adding SSc-specific characteristics to the risk stratification tools, and to identify the best risk assessment approach in SSc-PAH.

“The 2022 ESC/ERS Guidelines recommend upfront dual combination therapy for low- and intermediate-risk patients with SSc-PAH and triple therapy for high-risk patients. The efficacy of drugs has only been demonstrated for patients with mPAP ≥25 mmHg and PVR >3 WU. There is no treatment recommendation for patients with mPAP of 21–24 mmHg and PVR of 2–3 WU, and an individual assessment is advised for patients at high risk of PAH, such as SSc patients. Therefore, we assessed treatment regimens according to higher and lower mPAP and PVR thresholds, and risk stratification, and their association with mortality with the aim of optimizing the management of SSc-PAH patients.”

What are you working on next related to this research?
“Our research team at the Oslo University Hospital, led by Dr. Anna-Maria Hoffmann-Vold, is involved in a number of exciting projects. In particular, my PhD research focuses specifically on optimizing the management of SSc-PAH patients by evaluating risk stratification and treatment regimens using data from the EUSTAR cohort. We are also planning a home-monitoring project for our SSc-PAH patients in Norway, which is of particular importance due to the country’s long distances, in addition to the increasing use of digital e-health technologies and emphasis on self-management interventions and patient empowerment.”

What excites you most about your work?
“Being part of a national research team that focuses on SSc led by an international expert in the field, as well as taking part in numerous international collaborations, is exciting to me. Participating in research that will hopefully improve our SSc patients’ care is rewarding and meaningful. Research that is applicable to clinical practice stimulates my interest. Additionally, it is exciting to discover evidence of treatment benefits. The poor long-term survival of our SSc-PAH patients is, however, sobering. Hopefully, the prognosis of our SSc-PAH patients will improve through optimal risk stratification and aggressive treatment.”

What are you most looking forward to at ACR Convergence 2023 in San Diego?
“I am excited to present our work in the poster session and abstract session, to discuss our work, and to receive valuable feedback for our further projects. I look forward to networking opportunities and meetings with colleagues and experts in the field of my research that will allow discussions and the exchange of ideas. I also look forward to experiencing San Diego.”