Long-Term Prognostic and Hemodynamic Outcomes of Intensive Immunosuppressive Therapy in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

Abstract

Background

Intensive 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 Results

This 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).

Conclusions

This 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.