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

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 patients severe CTD-PAH. Methods The data 53 CTD-PAH hospitalized at Department Rheumatology and Immunology, Affiliated Drum Tower Hospital Nanjing University Medical School, were retrospectively reviewed. Patients followed up for an average 2 years track their outcomes. efficacy treatment survival rate determined. Results Among causes CTD-PAH, systemic lupus erythematosus (SLE) was most common (39.6%), age onset SLE-PAH younger than that other CTD. Bosentan more effective sildenafil reducing pulmonary artery pressure, improving cardiac function, increasing time. Combination therapy significantly improved prognosis compared monotherapy. Conclusions should be treated early vasodilators. In this study, bosentan superior sildenafil. Combined might option