Patterns of Pulmonary Arterial Pressure Trajectories Identified in Systemic Sclerosis

Five patterns of longitudinal pulmonary arterial pressure (PAP) changes were identified among patients with systemic sclerosis (SSc), according to study results published in RMD Open.

Data for this study were collected from 4 referral hospitals in Japan. Patients with SSc with 3 or more echocardiographic assessments of systolic PAP (sPAP) between 2008 and 2021 were included in the study. Longitudinal changes in PAP were evaluated using a group-based trajectory model.

The study population included 236 patients with 1097 sPAP measurements. Patients were aged a median of 61  years at SSc onset; 86% were women; 60% had anticentromere autoantibodies, 35% had lung fibrosis, 34% had hypertension, 25% had diffuse cutaneous SSc, and 7.6% had cardiac involvement.

Five trajectories were identified, including rapid progression (3.8%), early elevation (12.7%), middle elevation (22.9%), late elevation (10.2%), and low stable (50.4%).

In rapid progression, patients had an increase in systolic PAP soon after diagnosis; early elevation of PAP occurred within 5 years of diagnosis; middle elevation occurred after 5 years; late elevation occurred within 10 to 15 years; and in low stable, patients exhibited persistent, low PAP.

Compared with low stable, risk factors for rapid progression included cardiac involvement (odds ratio [OR], 28.85; 95% CI, 3.21-259.5) and age at onset (OR, 1.09; 95% CI, 1.00-1.18); for early elevation, they included diffuse cutaneous SSc (OR, 4.08; 95% CI, 1.27-13.12) and age at onset (OR, 1.08; 95% CI, 1.03-1.13), and for middle elevation, they included anticentromere autoantibodies (OR, 4.50; 95% CI, 1.11-18.19) and age at onset (OR, 1.04; 95% CI, 1.01-1.07).

During follow-up, 47 patients underwent right heart catheterization. In general, patients in groups with shorter time to PAP elevation had shorter pulmonary hypertension-free survival durations and shorter times to pulmonary hypertension, precapillary pulmonary hypertension, heart failure hospitalization, and all-cause mortality.

The results of the study may have been biased, as the timing of echocardiographic assessments was not protocolized.

The study authors concluded, “Depending on which trajectory the patient belonged to, there were differences not only in pulmonary hypertension-related clinical outcomes, but also in baseline clinical characteristics during the first [3] years after disease onset. Specifically, the rapid progression, early elevation, middle elevation and late elevation trajectories, in that order, showed earlier sPAP elevation, more frequent PH progression and poorer survival.”