Possible Protective Effect of Immunomodulatory Therapy on Development of Pulmonary Hypertension in Centromere Positive Systemic Sclerosis

Grace Alexander; Linder Wendt; Patrick Ten Eyck; Erin Sternhagen; Gulsen Ozen; Petar Lenert

doi:10.3390/immuno6010016

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Possible Protective Effect of Immunomodulatory Therapy on Development of Pulmonary Hypertension in Centromere Positive Systemic Sclerosis

Journal article

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Possible Protective Effect of Immunomodulatory Therapy on Development of Pulmonary Hypertension in Centromere Positive Systemic Sclerosis

Grace Alexander, Linder Wendt, Patrick Ten Eyck, Erin Sternhagen, Gulsen Ozen and Petar Lenert

Immuno, Vol.6(1), 16

03/10/2026

DOI: 10.3390/immuno6010016

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Abstract

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 (p = 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.

systemic sclerosis

centromere antibody

pulmonary hypertension

pulmonary arterial hypertension

Details

Title: Subtitle: Possible Protective Effect of Immunomodulatory Therapy on Development of Pulmonary Hypertension in Centromere Positive Systemic Sclerosis
Creators: Grace Alexander - University of Iowa
Linder Wendt - University of Iowa
Patrick Ten Eyck
Erin Sternhagen - Kaiser Permanente
Gulsen Ozen - University of Iowa
Petar Lenert - University of Iowa
Resource Type: Journal article
Publication Details: Immuno, Vol.6(1), 16
DOI: 10.3390/immuno6010016
ISSN: 2673-5601
eISSN: 2673-5601
Publisher: MDPI
Grant note: Kelting FoundationNational Center for Advancing Translational Sciences of the National Institutes of Health: UM1TR004403
Dr. Lenert's work was in part supported by a grant from the Kelting Foundation. Additionally, research reported in this publication was supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under Award Number UM1TR004403.
Language: English
Date published: 03/10/2026
Academic Unit: Biostatistics; Immunology; Internal Medicine; Design Biostat and Ethics
Record Identifier: 9985147182802771

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