Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease

Tobal, Rachid; Silvertand, D.; Gietema, Hester A.; Damoiseaux, Jan; Paassen, Pieter van

doi:10.1016/j.jtauto.2022.100174

“…This difference may be attributed to the various underlying systemic diseases present in our CTD-PAH patients. As SSc patients often exhibit subtle systemic immunological activity after the early in ammatory phase in comparison to conditions like SLE or rheumatoid arthritis, there is a potential for increased in ammation in the CTD-PAH group compared to our predominantly SSc based CTD patient cohort [50][51][52] . sICPs, far from being mere degradation by-products, actively modulate immune responses both locally and systemically.…”

Section: Discussionmentioning

confidence: 99%

Dephosphorylated uncarboxylated Matrix-Gla-Protein and vascular remodeling in pulmonary hypertension: an immunological connection

Tobal,

Potjewijd,

Vries

et al. 2024

Preprint

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Pulmonary arterial hypertension (PAH) is a disease characterized by pulmonary vascular remodeling. Since dephosphorylated-uncarboxylated Matrix Gla-Protein (dp-ucMGP) is associated with cardiovascular mortality in systemic sclerosis, a disease associated with PAH, and immune-system involvement in PAH is increasingly recognized, we investigated the relationship between dp-ucMGP, vascular remodeling and soluble immune-checkpoint proteins in PAH. This prospective cohort study included patients with idiopathic (I)PAH, connective tissue disease (CTD)-PAH, chronic thrombo-embolic PH (CTEPH) and CTD patients without PAH. Patients with IPAH and CTD-PAH were stratified by clinical signs of immune-mediated inflammatory disease (IMID). We measured dp-ucMGP plasma levels, soluble immune-checkpoint proteins (sICPs), and vascular smooth muscle cell (iVSMC) calcification. We found elevated dp-ucMGP levels in all PAH subtypes and CTD patients compared to healthy controls. PAH patients showed increased iVSMC calcification, but no direct correlation was found with dp-ucMGP. IMID-PAH patients had higher dp-ucMGP levels than non-IMID PAH patients. dp-ucMGP correlated with several sICPs in both IPAH and CTD patients; multiple sICPs were elevated in IMID PAH patients. High dp-ucMGP levels in IPAH patients were associated with worse survival. Our findings suggest dp-ucMGP as a potential biomarker of immune-mediated vascular remodeling in PAH. Hence, dp-ucMGP, could help identify PAH patients who might benefit from immunosuppressive therapies.

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A rare presentation of a young adult with Lambert-Eaton myasthenic syndrome and respiratory distress: Case report

van Doorn,

Huijs,

Timmermans

et al. 2024

Neuroimmunology Reports

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Dephosphorylated uncarboxylated Matrix-Gla-Protein as candidate biomarker for immune-mediated vascular remodeling and prognosis in pulmonary hypertension

Tobal,

Potjewijd,

de Vries

et al. 2024

Sci Rep

0

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Pulmonary arterial hypertension (PAH) is a disease characterized by pulmonary vascular remodeling. Since dephosphorylated-uncarboxylated Matrix Gla-Protein (dp-ucMGP) is associated with cardiovascular mortality in systemic sclerosis, a disease associated with PAH, and immune-system involvement in PAH is increasingly recognized, we investigated the relationship between dp-ucMGP, vascular remodeling and soluble immune-checkpoint proteins in PAH. This prospective cohort study included patients with idiopathic (I)PAH, connective tissue disease (CTD)-PAH, chronic thrombo-embolic PH (CTEPH) and CTD patients without PAH. Patients with IPAH and CTD-PAH were stratified by clinical signs of immune-mediated inflammatory disease (IMID). We measured dp-ucMGP plasma levels, soluble immune-checkpoint proteins (sICPs), and vascular smooth muscle cell (iVSMC) calcification. We found elevated dp-ucMGP levels in all PAH subtypes and CTD patients compared to healthy controls. PAH patients showed increased iVSMC calcification, but no direct correlation was found with dp-ucMGP. IMID-PAH patients had higher dp-ucMGP levels than non-IMID PAH patients. dp-ucMGP correlated with several sICPs in both IPAH and CTD patients; multiple sICPs were elevated in IMID PAH patients. High dp-ucMGP levels in IPAH patients were associated with worse survival. Our findings suggest dp-ucMGP as a potential biomarker of immune-mediated vascular remodeling in PAH. Hence, dp-ucMGP, could help identify PAH patients who might benefit from immunosuppressive therapies. Supplementary Information The online version contains supplementary material available at 10.1038/s41598-024-77000-w.

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Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease

Cited by 4 publications

References 24 publications

Dephosphorylated uncarboxylated Matrix-Gla-Protein and vascular remodeling in pulmonary hypertension: an immunological connection

Dephosphorylated uncarboxylated Matrix-Gla-Protein and vascular remodeling in pulmonary hypertension: an immunological connection

A rare presentation of a young adult with Lambert-Eaton myasthenic syndrome and respiratory distress: Case report

Dephosphorylated uncarboxylated Matrix-Gla-Protein as candidate biomarker for immune-mediated vascular remodeling and prognosis in pulmonary hypertension

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