Immuno · Published 2026-03-10 · DOI 10.3390/immuno6010016
The primary objective is to determine predictors of pulmonary hypertension (PH) development in patients with centromere antibody (ACA) positive systemic sclerosis (SSc) and to assess survival in patients with and without PH. This was a retrospective cohort study that included both prevalent and incident SSc patients with ACA. Clinical characteristics, mortality and immunomodulatory use were compared between SSc-ACA+ patients with and without PH. Univariable and multivariable logistic regression models, along with a univariable Cox proportional hazards model, were used to assess predictors and survival of PH, respectively. Of 146 SSc-ACA+ patients, 25 (17.1%) developed PH. Patients with PH had more frequent obstructive sleep apnea (36% vs. 12%), heart failure (44% vs. 7.4%), arrhythmias (32% vs. 12%), valvular heart disease (VHD) (32% vs. 8.3%), and chronic kidney disease (36% vs. 12%) than those without PH. In the multivariable logistic regression analysis, VHD was associated with an increased risk of PH development (OR = 7.79), while immunomodulatory use before PH was associated with a reduced risk of PH (OR = 0.34). Patients with PH who received immunomodulatory therapy had a better survival than those with PH without immunomodulatory treatment (<i>p</i> = 0.0008). PH is associated with high mortality in patients with SSc-ACA+. Immunomodulatory use may lower the incidence and mortality of PH in patients with SSc-ACA+ disease. Further randomized studies are needed to confirm this assumption.
Abstract from DOAJ. Public domain (CC0 1.0).
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