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Место терапии риоцигуатом у пациентов с легочной артериальной гипертензией, ассоциированной с системными заболеваниями соединительной ткани

https://doi.org/10.18087/cardio.2020.9.n1189

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Аннотация

Легочная гипертензия (ЛГ) может развиваться при различных системных аутоиммунных ревматических заболеваниях (САРЗ), таких как системная склеродермия (ССД), системная красная волчанка, ревматоидный артрит, смешанное заболевание соединительной ткани. Наиболее часто у пациентов с САРЗ развивается ЛГ первой группы по классификации ВОЗ (легочная артериальная гипертензия, ассоциированная с системными заболеваниями соединительной ткани; ЛАГ-СЗСТ). Общая распространенность данной патологии составляет до 15 случаев на 1 миллион взрослого населения. Большинство случаев ЛАГ-СЗСТ обусловлено ССД. Выживаемость больных ЛАГ-СЗСТ в целом ниже, чем выживаемость пациентов с другими формами ЛАГ. Лечение любого САРЗ, в том числе и при развитии ЛАГ, предполагает использование комплексного подхода с применением глюкокортикоидов, болезнь-модифицирующих препаратов течения ревматического заболевания (циклофосфамид, метотрексат, азатиоприн и др.) и генно-инженерных биологических препаратов. Большинству пациентов с ЛАГ-СЗСТ показано назначение специфической таргетной терапии. Представитель нового класса (стимуляторы растворимой гуанилатциулазы (рГЦ)) риоцигуат одобрен для лечения ЛАГ. Препарат обладает уникальным двойным механизмом действия: (1) сенсибилизация рГЦ к эндогенному оксиду азота (NO) путем стабилизации связи NO-рГЦ; (2) прямая NO-независимая стимуляция рГЦ. Для больных СЗСТ-ЛАГ риоцигуат является основной альтернативой ингибиторам фосфодиэстеразы-5, как в качестве монотерапии, так и в качестве комбинированной терапии.

Об авторах

Н. А. Шостак
ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н. И. Пирогова» Минздрава РФ, Москва, Россия
Россия
зав. кафедрой факультетской терапии им. акад. А.И. Нестерова, профессор


А. А. Клименко
ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н. И. Пирогова» Минздрава РФ, Москва, Россия
Россия
доцент кафедры факультетской терапии им. акад. А.И. Нестерова


Н. А. Демидова
ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н. И. Пирогова» Минздрава РФ, Москва, Россия
Россия
ассистент кафедры факультетской терапии им. академика А.И. Нестерова


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Для цитирования:


Шостак Н.А., Клименко А.А., Демидова Н.А. Место терапии риоцигуатом у пациентов с легочной артериальной гипертензией, ассоциированной с системными заболеваниями соединительной ткани. Кардиология. 2020;60(9):92-101. https://doi.org/10.18087/cardio.2020.9.n1189

For citation:


Shostak N.A., Klimenko A.A., Demidova N.A. The place of riociguat in the treatment of patients with pulmonary arterial hypertension associated with systemic connective tissue diseases. Kardiologiia. 2020;60(9):92-101. (In Russ.) https://doi.org/10.18087/cardio.2020.9.n1189

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