ArticleInternational journal of rheumatic diseases2025
Long-Term Prognostic and Hemodynamic Outcomes of Intensive Immunosuppressive Therapy in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease.
Article in International journal of rheumatic diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
2 citing papers in PubMed.
- Beyond Vasodilation: Sotatercept and the Dawn of Disease Modification in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.International journal of rheumatic diseases · 2026Article
- Long-Term Prognostic and Hemodynamic Outcomes of Intensive Immunosuppressive Therapy in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease.International journal of rheumatic diseases · 2025Article
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Authors and funding
15 authors.
Funding
Abstract
backgroundIntensive immunosuppressive therapy (IIT) is recommended for PAH associated with connective tissue disease (CTD-PAH). However, the long-term effects of IIT on pulmonary hemodynamics in this population remain unexplored. Additionally, its effectiveness in patients with systemic sclerosis (SSc)-associated PAH (SSc-PAH) is poorly understood. METHODS AND
resultsThis retrospective analysis included 69 consecutive patients with CTD-PAH treated at our institution (men/women: 9/60, mean age 55.3 ± 14.0 years). Patients were divided into two groups, wherein 41 patients received IIT (IIT group) and 28 did not (non-IIT group). Both groups received conventional vasodilator therapy. The prognosis and pulmonary hemodynamics were evaluated in all patients. The IIT group exhibited significantly lower rates of PAH-related mortality (p < 0.001) compared with the non-IIT group. The mean PAP (mPAP) improved significantly in the IIT group during the follow-up (baseline: 38.7 ± 12.2 mmHg; 1 year: 27.0 ± 8.2 mmHg; 5 years: 26.8 ± 7.3 mmHg, p < 0.05), while it remained unchanged in the non-IIT group. None of the patients with CTD-PAH required IIT retreatment. Among the 27 patients with SSc-PAH, the IIT group (n = 9) showed a significantly greater improvement in mPAP compared with the non-IIT group (n = 18) (ΔmPAP at 1 year: -13.4 ± 6.5 mmHg in IIT group vs. -3.0 ± 6.2 mmHg in non-IIT group, p < 0.001).
conclusionsThis study's findings suggest that IIT may lead to sustained improvements in pulmonary hemodynamics and better long-term outcomes in patients with CTD-PAH, including potential benefits in those with SSc-PAH.
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