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Pulmonary arterial hypertension (PAH) remains a serious disease characterized by elevated pulmonary artery pressure (PAP) and increased pulmonary vascular resistance (PVR). Among its subtypes, PAH associated with connective tissue disease (CPAH) has the worse prognosis, because of resistance to conventional vasodilator therapy. We hypothesized that intensive immunosuppressive therapy (IIT) could improve the pulmonary hemodynamics in CPAH.

circulation journal

Intensive Immunosuppressive Therapy Improves Pulmonary Hemodynamics and Long-Term Prognosis in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease