Consilium MedicumConsilium MedicumConsilium MedicumConsilium Medicum2075-17532542-2170Consilium Medicum9522710.26442/20751753.2019.12.190424ArticlesСтатьиReview ArticleENDOTHELIN RECEPTOR ANTAGONISTS IN PULMONARY HYPERTENSION TREATMENTМЕСТО АНТАГОНИСТОВ РЕЦЕПТОРОВ ЭНДОТЕЛИНА В ЛЕЧЕНИИ ЛЕГОЧНОЙ ГИПЕРТЕНЗИИShostakNadezhda A.ШОСТАКНАДЕЖДА АЛЕКСАНДРОВНАKlimenkoAlesia A.КЛИМЕНКОАЛЕСЯ АЛЕКСАНДРОВНАAndriyashkinaDarya lu.АНДРИЯШКИНАДАРЬЯ ЮРЬЕВНА

канд. мед. наук, доц. каф. факультетской терапии

andryashkina.darya@yandex.ruDemidovaNatalia A.ДЕМИДОВАНАТАЛЬЯ АЛЕКСАНДРОВНА

канд. мед. наук, ассистент каф. факультетской терапии

Pirogov Russian National Research Medical UniversityРоссийский национальный исследовательский медицинский университет имени Н. И. ПироговаPirogov Russian National Research Medical UniversityФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России151220192112VOL 21, NO12 (2019)ТОМ 21, №12 (2019)748028122021Copyright ©; 2019, Consilium MedicumCopyright ©; 2019, ООО "Консилиум Медикум"2019Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://consilium.orscience.ru/2075-1753/article/view/95227

The article presents new data on the use of endothelin receptor antagonists (ERA) in treatment of patients with different variants of pulmonary hypertension. The medications mechanisms of action are described and results of clinical studies on effectiveness and safety of the medications, perspectives on ERA use in clinical practice are presented. ERA is one of the best studied class of medications recommended for use in patients with idiopathic pulmonary arterial hypertension (PAH), PAH associated with generalized scleroderma and in Eisenmenger's syndrome in early stages of pulmonary hypertension. It was shown that this group of medications had a positive effect on functional, clinical and hemodynamic parameters, and increases life expectancy and quality of life in these serious cases. These medications are effective not only as monotherapy but also in combination treatment. Among medications for PAH treatment bosentan has the best evidence-based results according to the number of randomized clinical trials. It is the only medication from this group registered in Russia for children treatment. According to Russian register data among ERA in PAH patients 31.6% of patients use bosentan, 3.6% - ambrisentan, and 2.1% - macitentan. Bosentan is the second medication after sildenafil that is being used in PAH, in 31.6 and 54.5% of patients, respectively.

В статье представлены новые данные о применении антагонистов рецепторов эндотелина (АРЭ) в лечении больных различными вариантами легочной гипертензии. Описаны механизмы действия препаратов, приведены результаты клинических исследований по изучению их эффективности и безопасности, перспективы применения АРЭ в клинической практике. АРЭ являются одними из наиболее изученных классов препаратов, рекомендованных к применению у больных идиопатической легочной артериальной гипертензией (ЛАГ), ЛАГ вследствие системной склеродермии и при синдроме Эйзенменгера уже при начальных стадиях легочной гипертензии. Показано, что эта группа препаратов положительно влияет на функциональные, клинические, и гемодинамические параметры, увеличивает продолжительность и качество жизни этой тяжелой группы больных. Препараты эффективны как в монотерапии, так и при комбинированном лечении. По сравнению с другими ЛАГ-специфически-ми препаратами, наибольшую доказательную базу по количеству рандомизированных клинических исследований имеет бозентан, единственный в России препарат, который зарегистрирован в том числе для лечения детей. Согласно данным российского регистра больных ЛАГ среди АРЭ 31,6% больных получают бозентан, 3,6% - амбризентан, 2,1% - мацитентан. Бозентан занимает 2-е место по частоте назначения при ЛАГ после силденафила - 31,6 и 54,5% соответственно.

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