Легочная гипертензия при саркоидозе


https://doi.org/10.18093/0869-0189-2016-26-6-725-735

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Аннотация

Легочная гипертензия (ЛГ) является относительно частым осложнением саркоидоза. По разным данным, распространенность ЛГ при саркоидозе составляет 6–74 %. ЛГ, ассоциированная с саркоидозом (ЛГАС), как правило, связана с довольно плохим прогнозом. Несмотря на то, что ЛГАС чаще всего встречается у пациентов с тяжелыми стадиями саркоидоза, иногда она развивается при отсутствии поражения легочной паренхимы. Патофизиология развития ЛГАС довольно сложна и включает несколько различных механизмов, таких как легочный фиброз, гипоксическая вазоконстрикция, гранулематозная облитерация и / или ангиит легочных сосудов, внешняя компрессия легочных сосудов, легочная веноокклюзионная болезнь и поражение левых отделов сердца. Ведение пациентов с ЛГАС основано на устранении гипоксемии, лечении активного саркоидоза и сопутствующих заболеваний. Место системных глюкокортикостероидов в терапии ЛГАС остается противоречивым. Специфическая терапия легочной артериальной гипертензии (ЛАГ) пока не рекомендована для всех пациентов с ЛГАС, хотя в ряде небольших исследований у некоторых больных продемонстрирован положительный эффект применения простаноидов, антагонистов рецепторов эндотелина и ингибиторов фосфодиэстеразы5. Для подтверждения роли ЛАГспецифичной терапии у определенных групп больных ЛГАС необходимо проведение новых, хорошо спланированных рандомизированных плацебоконтролируемых исследований.


Об авторе

С. Н. Авдеев
Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии Федерального медико-биологического агентства России»
Россия

105077, Россия, Москва, ул. 11&я Парковая, 32, корп. 4

д. м. н., профессор, членкорр. Российской академии наук, руководитель клинического отдела Федерального государственного бюджетного учреждения «Научно-исследовательский институт пульмонологии Федерального медикобиологического агентства России»; тел.: (495) 4655264



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Дополнительные файлы

Для цитирования: Авдеев С.Н. Легочная гипертензия при саркоидозе.  Пульмонология. 2016;26(6):725-735. https://doi.org/10.18093/0869-0189-2016-26-6-725-735

For citation: Avdeev S.N. Pulmonary hypertension in sarcoidosis. Russian Pulmonology. 2016;26(6):725-735. (In Russ.) https://doi.org/10.18093/0869-0189-2016-26-6-725-735

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