Pulmonary Hypertension in Systemic Lupus Erythematosus: Current Evidence and Future Directions

Pulmonary hypertension (PH) is an uncommon but serious manifestation of systemic lupus erythematosus (SLE) that significantly impacts prognosis. Its subtle clinical presentation often overlaps with other cardiopulmonary complications, leading to underdiagnosis and delayed treatment. Immune-mediated endothelial injury, chronic inflammation, and progressive vascular remodeling contribute to increased pulmonary arterial pressure and right ventricular dysfunction. Advances in molecular and immunologic research have identified distinct biological profiles in SLE-associated PH, highlighting the roles of endothelial dysfunction, cytokine imbalance, and specific autoantibodies in disease progression. These insights support precision medicine approaches targeting both immune and vascular pathways. Early recognition and multidisciplinary management are essential, with echocardiography and right heart catheterization remaining fundamental for diagnosis, and biomarkers aiding in risk stratification. Treatment typically combines immunosuppressive and pulmonary vasodilator therapies, with corticosteroids and cyclophosphamide as first-line options, while biologic agents and targeted vasodilator combinations may further enhance clinical outcomes. SLE-associated PH exemplifies the intersection of autoimmunity and vascular disease, and integrating novel diagnostic and therapeutic strategies may improve early detection and survival outcomes.