Systemic sclerosis-associated pulmonary arterial hypertension is characterized by a distinct peripheral T helper cell profile.

Autor:	Papadimitriou TI; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands.; Department of Laboratory Medicine - Medical Immunology, Radboudumc, Nijmegen, The Netherlands., Lemmers JMJ; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., van Caam APM; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., Vos JL; Department of Cardiology, Radboudumc, Nijmegen, The Netherlands., Vitters EL; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., Stinissen L; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., van Leuven SI; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., Koenders MI; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., van der Kraan PM; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., Koenen HJPM; Department of Laboratory Medicine - Medical Immunology, Radboudumc, Nijmegen, The Netherlands., Smeets RL; Department of Laboratory Medicine - Medical Immunology, Radboudumc, Nijmegen, The Netherlands.; Radboudumc Laboratory for Diagnostics, Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, Netherlands., Nijveldt R; Department of Cardiology, Radboudumc, Nijmegen, The Netherlands., Vonk MC; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands., Thurlings RM; Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands.
Jazyk:	angličtina
Zdroj:	Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2024 Sep 01; Vol. 63 (9), pp. 2525-2534.
DOI:	10.1093/rheumatology/keae190
Abstrakt:	Objectives: Systemic sclerosis (SSc) is characterized by multiple clinical manifestations. Vasculopathy is a main disease hallmark and ranges in severity from an exacerbated Raynaud phenomenon to pulmonary arterial hypertension (PAH). The potential involvement of the immune system in SSc-associated vascular abnormalities is not clear. Here, we set out to study SSc-related immune parameters and determine whether and which peripheral T cell subsets associate with vascular severity in SSc patients. Methods: Peripheral blood and clinical data were collected from 30 SSc patients, 5 patients with idiopathic PAH and 15 age and sex-matched healthy donors (HD). In this cross-sectional cohort, SSc patients with PAH (n = 15) were matched for their age, sex and medication with SSc patients with no signs of PAH (n = 15). Lymphocyte subsets were quantified by multi-colour flow cytometry. Results: SSc patients exhibited elevated percentages of T peripheral helper cells (Tph), CD4+GZMB+ T cells and decreased levels of Th1 cells compared with HD. Increased presence of both CD4+ and CD8+ exhausted-like (CD28-) T cells, characterized by raised cytokine and cytotoxic signature, was also observed in SSc compared with HD blood. Furthermore, IL-4 expressing CD4+CD8+ T cells were significantly increased in SSc peripheral blood. Interestingly, the presence of PAH in SSc was accompanied by a distinct T helper profile, characterized by raised percentages of Th17 and Tph cells. Conclusion: SSc patients with severe vasculopathy (presence of PAH) exhibited a distinct T cell profile, suggesting a potential role of auto-immune inflammation in SSc vascular complications. (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology.)
Databáze:	MEDLINE
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