This study illustrated distinct cytokine profiles that can distinguish SSc patients who are at high-risk for or have PAH from SSc patients who are at low risk for PAH and HC. We anticipate these data could be used to risk stratify SSc patients and guide therapy. These data may provide potential mechanistic targets to increase our understanding of SSc-PAH and support therapeutic innovation in the future.
SSc: Systemic sclerosis; PAH: Pulmonary arterial hypertension; PHAROS: Pulmonary hypertension assessment and recognition of outcomes in scleroderma; HC: Healthy controls; RHC: Right heart catheterization; mPAP: Mean pulmonary artery pressure; PCWP: Pulmonary capillary wedge pressure; FVC: Forced vital capacity; DLCO: Diffusing capacity of carbon monoxide; RVSP: Right ventricular systolic pressure; FDR: False discovery rate.
The authors would like to thank all the investigators and patients who participated in the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry.
Financial support for this study was received from the Scleroderma Research Foundation and Translational Research and Applied Medicine (TRAM) Pilot Grant Program.
Availability of data and materials
The datasets generated during the current study is publicly available from the NCBI. The GEO accessions numbers are GSE195581 and GSE195583.
Ethics approval and consent to participate
The Institutional Review Board at 22 participating US centers approved the PHAROS protocol and patients provided written informed consent prior to enrollment.
Consent for publication
Arthritis Res Ther. 2022;24(39) © 2022 BioMed Central, Ltd.
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