PULMONARY ARTERIAL HYPERTENSION-TARGETED THERAPIES IN PATIENTS WITH CONNECTIVE TISSUE DISEASE-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION: A SYSTEMATIC REVIEW AND NETWORK META-ANALYSIS

Abstract

Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is the most severe form and has the lowest survival rates among all pulmonary arterial hypertension (PAH) subgroups. However, no consensus exists on the most effective therapy in reducing the risk of clinical worsening for this patient population. This systematic review and network meta-analysis aimed to identify the most effective PAH-targeted treatment for CTD-PAH patients. Seven randomized controlled trials consisting of 1,042 patients were identified by MEDLINE, Scopus, and ClinicalTrials.gov. Combination therapy with an endothelin receptor antagonist (ERA) and a phosphodiesterase-5 inhibitor (PDE5i) significantly reduced the risk of clinical worsening compared to placebo (hazard ratio, 0.31; 95% confidence interval, 0.13 to 0.74). Moreover, combination therapy ranked highest for reducing the clinical worsening, followed by PDE5i and ERA monotherapies. Based on these findings, combination therapy is the preferred treatment for patients with CTD-PAH. These findings provide valuable insights into clinical decision-making and help tailor therapies for this patient group, who may have diverse treatment responses compared to those with idiopathic PAH.