Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases

Abstract Background Pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD) (CTD‐PAH) remains a difficult challenge in clinical practice. We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD‐PAH. Methods The data of 53 patients with severe CTD‐PAH hospitalized at the Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, were retrospectively reviewed. Patients were followed up for an average of 2 years to track their outcomes. The efficacy of treatment and the survival rate of patients with severe CTD‐PAH were determined. Results Among the causes of severe CTD‐PAH, systemic lupus erythematosus (SLE) was the most common (39.6%), and the age at onset in patients with SLE‐PAH was younger than that of patients with other CTD. Bosentan was more effective than sildenafil in reducing pulmonary artery pressure, improving cardiac function, and increasing survival time. Combination therapy with targeted vasodilators significantly improved the prognosis of patients with severe CTD‐PAH compared with monotherapy. Conclusions Patients with severe CTD‐PAH should be treated early with targeted vasodilators. In this study, bosentan was superior to sildenafil. Combined treatment might be an option for severe CTD‐PAH.