rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2025-262-272rsp-3759Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL RESEARCHЛегочная артериальная гипертензия, ассоциированная с системной склеродермией: Результаты одноцентрового длительного проспективного наблюдения 2009–2024 гг.Pulmonary arterial hypertension associated with systemic sclerosis: Results of a single-center long-term follow-up in 2009–2024https://orcid.org/0000-0003-1784-3699ВолковА. В.VolkovA. V.

Волков Александр Витальевич.

115522, Москва, Каширское шоссе, 34а

Alexander V. Volkov.

115522, Moscow, Kashirskoye Highway, 34A

sandyvlk@yahoo.comhttps://orcid.org/0000-0001-8469-8423ЮдкинаН. Н.YudkinaN. N.

115522, Москва, Каширское шоссе, 34а

Natalia N. Yudkina.

115522, Moscow, Kashirskoye Highway, 34A

https://orcid.org/0000-0002-6906-0621НиколаеваЕ. В.NikolaevaE. V.

115522, Москва, Каширское шоссе, 34а

Ekaterina V. Nikolaeva.

115522, Moscow, Kashirskoye Highway, 34A

https://orcid.org/0000-0002-1598-8360НасоновЕ. Л.NasonovE. L.

115522, Москва, Каширское шоссе, 34а

Evgeny L. Nasonov.

115522, Moscow, Kashirskoye Highway, 34A

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation202502072025633262272Copyright © Волков А.В., Юдкина Н.Н., Николаева Е.В., Насонов Е.Л., 20252025Волков А.В., Юдкина Н.Н., Николаева Е.В., Насонов Е.Л.Volkov A.V., Yudkina N.N., Nikolaeva E.V., Nasonov E.L.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/3759

Цель исследования – анализ выживаемости и влияния на нее этиологии, факторов неблагоприятного прогноза и терапии у пациентов с легочной артериальной гипертензией (ЛАГ), ассоциированной с системной склеродермией (ССД).

Материал и методы

Материал и методы. Наблюдались 76 больных ССД с ЛАГ, которым назначалась ЛАГ-специфическая терапия, с последующим мониторированием эффективности и переносимости не менее 5 лет. Группа сопоставления («исторический контроль») – 20 пациентов с ССД, наблюдавшихся в годы, когда ЛАГ-специфическая терапия была не доступна. Конечная точка исследования – смерть от всех причин.

Результаты

Результаты. Применение ЛАГ-специфической терапии на 67% снижает 5-летний риск смерти больных ССД с ЛАГ в сравнении с «историческим контролем». На современном этапе 1-, 2-, 3- и 5-летняя выживаемость при ССД с ЛАГ составила 88%, 76%, 68% и 51% соответственно. В качестве факторов, ассоциированных с летальностью, определялись возраст, функциональный класс, дистанция теста 6-минутной ходьбы, давление в правом предсердии, сердечный выброс, легочное сосудистое сопротивление, повышение уровня биомаркеров. Применение мацитентана и/или риоцигуата в виде монотерапии или в комбинации с другим ЛАГ-специфическим препаратом статистически значимо снижало 5-летний риск наступления летального исхода (относительный риск – 0,44; 95%-й доверительный интервал: 0,19–1,07; р=0,07). Иммуносупрессивная терапия влияния на выживаемость не оказывала.

Заключение

Заключение. Выживаемость больных ССД с ЛАГ остается низкой, необходимы дальнейшие исследования, направленные на поиск новых патогенетических мишеней; применение современных ЛАГ-специфических препаратов (мацитентан и/или риоцигуат) модифицирует течение болезни, увеличивая выживаемость.

The aim of this study was to investigate the survival and the impact of various factors on it in patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (PAH-SSc).

Methods

Methods. We analyzed the data of 76 patients diagnosed with PAH-SSc who received PAH-specific therapy and were followed-up for at least 5 years. A group of “historical control” consisted of 20 patients who did not receive PAH-specific treatment. The primary endpoint of the study was death from any cases.

Results

Results. The use of PAH-specific therapy significantly reduces the 5-year risk of death in patients with PAH-SSc by 67%, compared to the “historical control” group. At the present time, 1-, 2-, 3-, and 5-year survival rates for patients with PAH-SSc are 88%, 76%, 68%, and 51%, respectively. Factors associated with mortality include age, functional class, 6-minute walking distance, right atrial pressure, cardiac output, pulmonary vascular resistance, and elevated biomarkers. The use of macitentan and/or riociguat as monotherapy or in combination with other PAH-specific medications significantly reduced the risk of death after 5 years (hazard ratio – 0.44 [0.19; 1.07]; p=0.07).

However, immunosuppressive therapy did not improve survival.

Conclusion

Conclusion. The survival rate for patients with PAH-SSc remains low. Further research is needed to identify new treatments targets. The use of modern PAH-specific drugs, such as macitentan and riociguat, can modify the course of disease and improve survival.

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The authors declare that there are no conflicts of interest present.