ESC scoring tool predicts survival in SSc-associated pulmonary arterial hypertension
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Key takeaways:
- The updated European Society of Cardiology risk assessment tool accurately estimated mortality risk among patients with SSc-PAH.
- Non-invasive variables allow “accessible means to guide treatment and predict outcome,” the researchers wrote.
The 2022 European Society of Cardiology risk assessment tool, when applied at baseline and follow-up, can accurately predict survival in patients with systemic sclerosis-associated pulmonary arterial hypertension, according to data.
“In 2015, the European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines recommended a multidimensional risk assessment tool to guide treatment decisions and prognostication in pulmonary arterial hypertension (PAH),” Zoe Brown, MBBS, FRACP, of St. Vincent’s Hospital in Melbourne, Australia, and colleagues wrote in Arthritis Care & Research. “The 2015 ESC risk assessment tool stratified prognostic variables according to cut-points as having low, intermediate or high risk of one-year mortality.
“A criticism of the 2015 ESC risk score is that the majority of Group 1 PAH and SSc-PAH patients are classified in the intermediate stratum at baseline and at follow up,” they added. “The latest 2022 ESC/ERS guidelines recommend a more nuanced risk stratification assessment to better characterize those in the intermediate risk profile during follow up.”
According to Brown and colleagues, the new, simplified tool is based on three variables — WHO functional class, serum N-terminal pro-brain type natriuretic peptide (NT-proBNP), and six-minute walk distance, applied during follow up visits. To validate the 2022 ESC risk prediction tool, the researchers retrospectively analyzed data from two prospective studies: the Systemic Sclerosis Cohort Singapore and the Australian Scleroderma Cohort Study. The analysis included 260 patients with incident SSc-PAH, with 54 from Singapore and 206 from Australia.
At baseline, patients were classified as having low, intermediate or high risk for death. Later, at follow-up — defined as the first annual registry visit within 2 years of diagnosis — there were four strata: low, intermediate-low, intermediate-high and high risk. The relationship between score and risk for death was estimated using Kaplan-Meier curves, Cox hazards regression and accelerated failure time (AFT) models, which assume “that any time spent in a higher risk profile accelerates time to mortality,” the researchers wrote.
According to the researchers, the 2022 tool graded 72.2% of patients with SSc-PAH as having an intermediate risk for death at baseline, while 55.5% were rated as low or intermediate-low risk at follow-up. Higher risk, based on the baseline three-strata model, was associated with increased all-cause and cardiac-cause mortality, while the four-strata model at follow-up demonstrated statistically significant increases in all-cause and cardiac mortality with higher risk score, the researchers wrote.
“Our results, strengthened by the inclusion of AFT modeling, confirm the importance of achieving a low-risk status in SSc-PAH in order to improve survival,” Brown and colleagues wrote. “This can be done through regular risk assessments, which enable the appropriate guidance on the use and escalation of PAH-specific therapy in accordance with the 2022 ESC guidelines. For rheumatologists who may be the primary physicians in the care of SSc patients, the non-invasive variables required to complete the 2022 four-strata risk assessment at follow-up provide an accessible means to guide treatment and predict outcome.”
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