Легенева гіпертензія, асоційована із системною склеродермією (огляд літератури)
DOI:
https://doi.org/10.30978/TB2025-3-87Ключові слова:
легенева гіпертензія; легенева артеріальна гіпертензія; системна склеродермія.Анотація
Легенева гіпертензія (ЛГ) є однією з провідних причин захворюваності та смертності при системній склеродермії (ССД).
Мета роботи — провести аналіз сучасних літературних даних щодо епідеміології, механізмів розвитку, ризику, клінічних виявів, діагностики, лікування та прогнозу в пацієнтів із ССД та ЛГ різних груп.
Матеріали та методи. Проаналізовано опубліковані за останніх 15 років (2010—2025) статті з баз даних Scopus, MEDLINE, PubMed. Для остаточного аналізу відібрано 71 статтю, опубліковану англійською мовою.
Результати та обговорення. Легеневу гіпертензію діагностують у 7—31 % пацієнтів із ССД. При ССД може розвиватися ЛГ різних груп: легенева артеріальна гіпертензія (ЛАГ), асоційована із системними захворюваннями сполучної тканини, ЛАГ з ознаками ураження вен/капілярів, ЛГ, асоційована із захворюваннями лівих відділів серця, ЛГ, асоційована із захворюванням легень, і хронічна тромбоемболічна ЛГ. Легенева артеріальна гіпертензія розвивається в 12,9—19,7 % пацієнтів. Незважаючи на таргетну терапію, вона залишається основною причиною смертності пацієнтів із ССД. Діагностика ЛГ може бути утруднена через гетерогенність клінічних виявів ССД і неспецифічну клінічну картину ЛГ на ранніх стадіях, що призводить до пізньої діагностики. Для своєчасної діагностики розроблені алгоритми скринінгу ЛАГ, зокрема алгоритми DETECT і ASIG, що дають змогу відібрати пацієнтів для катетеризації правих відділів серця.
Висновки. Легенева гіпертензія є тяжким виявом/ускладненням ССД, що асоціюється з несприятливим прогнозом і погіршенням якості життя пацієнтів. При ССД ЛГ є гетерогенним станом (ЛГ різних груп). Рання діагностика та лікування ЛГ поліпшують прогноз хворих. Пацієнтам із ССД рекомендований скринінг на наявність ЛАГ, оскільки раннє її виявлення дасть змогу зменшити гемодинамічні порушення та поліпшити виживаність.
Посилання
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