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A study presented at the American College of Rheumatology annual meeting assessed the performance of current and proposed risk stratification criteria to estimate 1- and 3- year mortality for patients with newly diagnosed systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH).

In this exclusive 番茄社区 video, Hilde Jenssen Bjørkekjær, MD, from the department of rheumatology at the Hospital of Southern Norway in Kristiansand, discusses , which looked at patients from 2001 to 2021 in the European Scleroderma Trial and Research (EUSTAR) database.

Following is a transcript of her remarks:

Pulmonary arterial hypertension is an obliterative vasculopathy that leads to increased pulmonary vascular resistance that leads to right heart failure and death, if not treated. PAH develops in 6-7% of patients with systemic sclerosis and is one of the major leading causes of death in these patients, with a median survival of 3 to 4 years.

The 2022 ESC/ERS [European Society of Cardiology/European Respiratory Society] guidelines for diagnosis and treatment of pulmonary hypertension recommend risk stratification to classify patients of low, intermediate, or high risk for deaths with an estimated 1-year mortality of less than 5%, 5-20%, and greater than 20%, respectively. And this also is used to guide optimized management.

And in the same guidelines, they also publish the new definition of pre-capillary pulmonary hypertension, which is a mean pulmonary arterial pressure (mPAP) >20 mm Hg, a wedge pressure ≤15 mm Hg, and a pulmonary vascular resistance >2.

Several risk stratification approaches have been developed for PAH but mainly derived from idiopathic pulmonary arterial hypertension. And as we know, PAH associated with systemic sclerosis has a worse prognosis than idiopathic PAH, even if they may appear at the lower risk level at baseline with milder hemodynamic impairment. So it's important to find the best risk assessment approach for SSc-PAH to capture this increased risk to optimize the management of these patients.

We wanted to assess some of the current risk stratification tools to estimate the 1- and 3- year mortality and to assess this in both patients with mPAP ≥25, which was the old criteria. And for the patients with mPAP 21 to 24 mm Hg.

We used the data from the EUSTAR database, the European Scleroderma Trial and Research Group, which includes more than 20,000 patients from more than 200 international centers. And of 955 patients who underwent right heart catheterization, 422 had PAH according to the new definition -- 342 with mPAP ≥25 and 80 patients with mPAP between 21 and 24.

And we applied four different models for risk stratification at baseline, using risk parameters with values based on the guidelines. And of the models that seemed to perform best to estimate the mortality in these patients that was based on the ESC/ERS guidelines, with inspiration from the Swedish PAHR [Pulmonary Arterial Hypertension Registry] group and the German COMPERA group, where we used as many risk parameters as possible from the risk table in the guidelines. And each of the variables were graded from one to three, representing low to high risk. And the mean of available risk parameters were used to classify the patients into low, intermediate, and high risk. We also tried to segregate the intermediate-risk group into intermediate-low and intermediate-high, since previous studies have shown differences in survival in these two groups.

So using this group, we found that most patients with mPAP ≥25 were diagnosed at an intermediate-risk level, whereas most patients with mPAP 21 to 24 were diagnosed at the low-risk level. And for the group with mPAP ≥25, the three-strata version, which stratified patients into low, intermediate, and high risk, stratified patients according to the estimated 1-year mortality, as suggested by the guidelines, with a 1-year mortality of 4.7% for the low-risk group, 11% for the intermediate-risk group, and 40% for the high-risk group. And the four-strata version, which also segregated into intermediate risk group, segregated the mortality further in this group with a 1-year mortality of 8% for the intermediate-low-risk group, and 17% for the intermediate-high-risk group.

But for mPAP 21 to 24, all models performed poorly. Only one of these patients died, and this patient was stratified as low-risk in all the models. And when we looked at the 3-year mortality, it was even clearer that the intermediate-high-risk group in the patients with mPAP ≥25 had a 3-year mortality closer to the high-risk group with a 3-year mortality of 58% in the intermediate-high-risk group, and 62% in the high-risk group.

So we concluded that for patients with mPAP ≥25, that model segregated 1-year mortality according to the guidelines, and that it seems useful to sub-stratify the intermediate-risk group to identify the patients with the intermediate-high risk that has a particularly high mortality closer to the high=risk group. And that the patients with mPAP 21 to 24 were diagnosed at the lower risk level, but the 3-year mortality was nevertheless high at 12%. But the risk stratification models estimated the mortality poorly.

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