pmedpharmФармация и фармакологияPharmacy & Pharmacology2307-92662413-2241Pyatigorsk Medical and Pharmaceutical Institute - branch of Volgograd State Medical Univer10.19163/2307-9266-2024-12-5-338-349pmedpharm-1663Research ArticleОБЗОРЫREVIEWПодходы к лечению лимфангиолейомиоматоза лёгких: от радикальной хирургии до молекулярной биологииTreatment approaches to pulmonary lymphangioleomyomatosis: From surgical extirpation to molecular biologyhttps://orcid.org/0000-0002-8633-8496ПоловниковИ. В.PolovnikovI. V.

врач-пульмонолог, старший лаборант кафедры патологической анатомии с патологоанатомическим отделением, ФГБОУ ВО ПСПбГМУ им. И.П. Павлова Минздрава России.

197022, Россия, Санкт-Петербург, ул. Льва Толстого, д. 6–8.

pulmonologist, senior laboratory assistant of the department of pathological anatomy with a pathology clinical department, Pavlov First Saint Petersburg State Medical University. 

Bld. 6, 8, Lev Tolstoy Str., Saint Petersburg, Russia, 197022.

policrats@mail.ruhttps://orcid.org/0000-0001-8888-4135ЮкинаГ. Ю.YukinaG. Yu.

кандидат биологических наук, доцент, заведующий научной лабораторией патоморфологии научно-клинического центра патоморфологии, ФГБОУ ВО ПСПбГМУ им. И.П. Павлова Минздрава России. 

197022, Россия, Санкт-Петербург, ул. Льва Толстого, д. 6–8.

Candidate of sciences (Biology), associate professor, head of the scientific laboratory of pathomorphology of the scientific and clinical center of pathomorphology, Pavlov First Saint Petersburg State Medical University.

Bld. 6, 8, Lev Tolstoy Str., Saint Petersburg, Russia, 197022.

pipson@inbox.ruhttps://orcid.org/0000-0001-5521-7248СухоруковаЕ. Г.SukhorukovaE. G.

кандидат медицинских наук, старший научный сотрудник научной лаборатории патоморфологии научно-клинического центра патоморфологии, ФГБОУ ВО ПСПбГМУ им. И.П. Павлова Минздрава России. 

197022, Россия, Санкт-Петербург, ул. Льва Толстого, д. 6–8.

Candidate of sciences (Medicine), senior researcher at the scientific laboratory of pathomorphology of the scientific and clinical center of pathomorphology, Pavlov First Saint Petersburg State Medical University.

Bld. 6, 8, Lev Tolstoy Str., Saint Petersburg, Russia, 197022.

len48@inbox.ruФедеральное государственное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный медицинский университет им. академика И.П. Павлова» Министерства здравоохранения Российской ФедерацииРоссияPavlov First Saint Petersburg State Medical University.Russian FederationФедеральное государственное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный медицинский университет им. академика И.П. Павлова» Министерства здравоохранения Российской Федерации.РоссияPavlov First Saint Petersburg State Medical University.Russian Federation202429062025125338349Copyright © Половников И.В., Юкина Г.Ю., Сухорукова Е.Г., 20252025Половников И.В., Юкина Г.Ю., Сухорукова Е.Г.Polovnikov I.V., Yukina G.Y., Sukhorukova E.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.pharmpharm.ru/jour/article/view/1663Цель

Цель. Сбор и систематизация данных о подходах к лечению лимфангиолейомиоматоза (ЛАМ) лёгких, основанных на представлениях о патогенезе заболевания.

Материалы и методы

Материалы и методы. Проанализировано 1157 статей и монографий (включая дубли), из которых отобраны 70 оригинальных источников. Поиск источников осуществляли в базах PubMed, eliabrary.ru, CyberLeninka. Глубина поиска составила 50 лет — с 1973 г. по август 2023 г. Авторы акцентировали внимание на актуальных публикациях в высокорейтинговых научных журналах.

Результаты

Результаты. В обзоре представлены подходы к лечению ЛАМ, основанные как на клинических наблюдениях за течением заболевания, так и на экспериментальных данных о вероятном его патогенезе. Собранные данные представлены в хронологическом порядке, начиная с радикальных методов, основанных на представлениях о безусловной связи развития ЛАМ с воздействием женских половых гормонов. Отдельное внимание уделено препаратам из группы ингибиторов mTOR, в том числе их профилю безопасности. Помимо этого, приведены результаты исследований, демонстрирующих новые перспективные методы лекарственной терапии ЛАМ, сочетающие в себе как применение ингибиторов mTOR с другими препаратами, так и основанных на изолированном использовании альтернативных групп медикаментов.

Заключение

Заключение. Используемые на настоящий момент методы лекарственной терапии и предлагаемые новые методы направлены лишь на лечение уже сформировавшегося заболевания, а проведение эффективной медикаментозной профилактики ЛАМ сейчас представляется практически невозможным ввиду отсутствия полного понимания патогенеза заболевания и, что более важно, — его этиологии. Данный вопрос является наиболее актуальным в определении дальнейших перспектив развития фармакотерапевтических подходов к ЛАМ.

The aim of the work was to collect and systematize the data on the treatment approaches to pulmonary lymphangioleiomyomatosis (LAM) based on insights into the pathogenesis of the disease.

Materials and methods

Materials and methods. 70 original sources have been selected from analyzed 1 157 articles and monographs (including duplicates). The search for the sources was carried out in the databases of PubMed, eLibrary.ru, Cyberleninka for a fifty-year period of publications (from 1973 to August 2023), with an emphasis on more current publications and the ones in highly rated scientific journals.

Results

Results. The review presents the treatment approaches to LAM, based both on clinical observations of the disease course and on the experimental data on its probable pathogenesis. The collected data are presented in the chronological order, starting from radical methods based on the idea of an unconditional connection between the development of LAM and the female sex hormones. Special attention has been paid to the drugs from the group of mTOR inhibitors, including their safety profile. In addition, the results of the studies demonstrating new promising methods of the LAM drug therapy, both combining the use of mTOR inhibitors with other drugs, and the ones based on the isolated use of alternative groups of drugs, are presented in the work.

Conclusion

Conclusion. The currently used methods of the drug therapy and the proposed new methods are aimed at only treating an already established disease, and the effective drug prevention of LAM now seems almost impossible due to the lack of a complete understanding about its pathogenesis and, more importantly, its etiology. This issue is the most relevant in determining further prospects for the development of pharmacotherapeutic approaches to LAM.

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