Abstract
Pulmonary arterial hypertension associated with connective tissue diseases is a significant medical problem characterized by complex clinical manifestations and requiring a special approach in treatment. Standard treatments are often ineffective and cause side effects. Many patients suffering from systemic lupus erythematosus and scleroderma face this condition, which highlights the need to find new therapeutic solutions. Materials and methods. The study was conducted in the Department of Arterial Hypertension of the Republican Clinical Hospital of Makhachkala from January to September 2024. Patients aged 18 to 70 years were randomized and were treated with a combination of ambrisentan and tadalafil or one of these drugs in combination with placebo. The main inclusion criteria were mean pulmonary artery pressure ≥ 25 mmHg and pulmonary vascular resistance ≥ 240 dynes· s/cm5. Outcomes were assessed by time to first clinical failure and secondary measures such as NT-proBNP level and 6-minute walk test. Results. A total of 23 of 43 patients had DST-Lag. Combination therapy demonstrated a significant 71% reduction in the risk of clinical failure compared to monotherapy. Patients on combination therapy walked an average of 380 meters in 6 minutes, which is comparable to the results on monotherapy. Conclusions. Combination therapy was found to be more effective in reducing the risk of adverse clinical outcomes and was associated with manageable side effects. The results highlight the importance of considering individual disease characteristics when choosing therapy.
Published Version
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