Recent Advances in Targeted Therapy for Connective Tissue Disease-associated Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a common and severe vascular complication in patients with connective tissue diseases (CTDs), and it also represents a leading cause of adverse outcomes and mortality in this population. Over the past decades, the continuous emergence of effective therapeutic approaches targeting the key signaling pathways of PAH has significantly alleviated patients’ symptoms and improved their quality of life. As a crucial subtype of PAH, patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) have also gained substantial benefits from these advances. Targeted therapy serves as the core treatment modality, and in recent years, numerous breakthroughs have been achieved in the field of CTD-PAH targeted therapy. This article systematically reviews the latest research progress of both traditional and emerging targeted therapies for CTD-PAH.