pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2026-36-5-871-882pulmo-5040Research ArticleКЛИНИЧЕСКАЯ ФАРМАКОЛОГИЯCLINICAL PHARMACOLOGYТройная комбинация ивакафтор + тезакафтор + элексакафтор / ивакафтор при муковисцидозе: клиническая эффективность и безопасность оригинального препарата и его генерического аналога Трилекса®Triple combination ivacaftor + tezacaftor + elexacaftor and ivacaftor for cystic fibrosis: clinical efficacy and safety of the original drug and its generic analogue Trilexa®https://orcid.org/0000-0002-3174-5000МержоеваЗ. М.MerzhoevaZ. M.

Мержоева Замира Магомедовна – д. м. н., доцент кафедры пульмонологии Института клинической медицины имени Н.В.Склифосовского, заведующая пульмонологическим отделением Университетской клинической больницы № 4

119991, Москва, ул. Трубецкая, 8, стр. 2 тел.: (495) 708-35­-76 

Zamira M. Merzhoeva, Doctor of Medicine, Associate Professor, Department of Pulmonology, N.V.Sklifosovsky Institute of Clinical Medicine; Head of the Pulmonology Department, University Clinical Hospital No.4

ul. Trubetskaya 8, build. 2, Moscow, 119991  tel.: (495) 708­-35­76 

zamira.merzhoeva@bk.ruhttps://orcid.org/0000-0002-0928-2900ЛевинаЮ. А.LevinaI. A.

Левина Юлия Алексеевна – аспирант кафедры пульмонологии Института клинической медицины имени Н.В.Склифосовского

119991, Москва, ул. Трубецкая, 8, стр. 2 тел.: (495) 708-­35-76 

Iuliia A. Levina, Resident, Department of Pulmonology, N.V.Sklifosovsky Institute of Clinical Medicine

ul. Trubetskaya 8, build. 2, Moscow, 119991 tel.: (495) 708­35­-76

yu1999levina@gmail.comhttps://orcid.org/0000-0003-1254-6863ЛавгиноваБ. Б.LavginovaB. B.

Лавгинова Баина Баатровна – аспирант кафедры пульмонологии Института клинической медицины имени Н.В.Склифосовского

119991, Москва, ул. Трубецкая, 8, стр. 2 тел.: (495) 708-35­-76 

Baina B. Lavginova, Resident, Department of Pulmonology, N.V.Sklifosovsky Institute of Clinical Medicine

ul. Trubetskaya 8, build. 2, Moscow, 119991 tel.: (495) 708-­35-76

bapus15@yandex.ruhttps://orcid.org/0000-0001-9661-7213СувороваО. А.SuvorovaO. A.

Суворова Ольга Александровна – ассистент кафедры пульмонологии Института клинической медицины имени Н.В.Склифосовского

119991, Москва, ул. Трубецкая, 8, стр. 2тел.: (495) 609­-14-­00

Olga A. Suvorova, Assistant, Pulmonology Department, N.V.Sklifosovsky Institute of Clinical Medicine

ul. Trubetskaya 8, build. 2, Moscow, 119991  tel.:(495) 609­14­-00

Olga.a.suvorova@mail.ruhttps://orcid.org/0000-0003-0503-6371КаширскаяН. Ю.KashirskayaN. Yu.

Каширская Наталия Юрьевна – д. м. н., профессор, главный научный сотрудник лаборатории генетической эпидемиологии

Scopus ID: 6507308033; WoS Researcher ID: C­6404­2012; РИНЦ ID: 93625

115522, Москва, ул. Москворечье, 1 тел.: (499) 324-­12-­24

Nataliya Yu. Kashirskaya, Doctor of Medicine, Professor, Chief Researcher, Laboratory of Genetic Epidemiology

Scopus ID: 6507308033; WoS Researcher ID: C­6404­2012; РИНЦ ID: 93625 

ul. Moskvorechye 1, Moscow, 115522  tel.: (499) 324­-12-­24 

kashirskayanj@mail.ruhttps://orcid.org/0000-0002-5999-2150АвдеевС. Н.AvdeevS. N.

Авдеев Сергей Николаевич – д. м. н., профессор, академик Российской академии наук; директор Национального медицинского исследовательского центра по профилю «Пульмонология»; заведующий кафедрой пульмонологии Института клинической медицины имени Н.В.Склифосовского; главный внештатный пульмонолог Министерства здравоохранения Российской Федерации

SciProfiles: 741582; Scopus Author ID: 7003292838

119991, Москва, ул. Трубецкая, 8, стр. 2 тел.: (495) 708-35-­76 

Sergey N. Avdeev, Doctor of Medicine, Professor, Academician of Russian Academy of Sciences, Director of the National Medical Research Center for Pulmonology; Head of the Department of Pulmonology, N.V.Sklifosovsky Institute of Clinical Medicine; Chief Pulmonologist of the Ministry of Health of the Russian Federation

SciProfiles: 741582; Scopus Author ID: 7003292838

ul. Trubetskaya 8, build. 2, Moscow, 119991 tel.: (495) 708­35-­76 

serg_avdeev@list.ruФедеральное государственное автономное образовательное учреждение высшего образования Первый Московский государственный медицинский университет имени И.М.Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет)РоссияFederal State Autonomous Educational Institution of Higher Education I.M.Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)Russian FederationФедеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н.П.Бочкова» Министерства науки и высшего образования Российской ФедерацииРоссияResearch Centre for Medical GeneticsRussian Federation202608102026365871882Copyright © Мержоева З.М., Левина Ю.А., Лавгинова Б.Б., Суворова О.А., Каширская Н.Ю., Авдеев С.Н., 20262026Мержоева З.М., Левина Ю.А., Лавгинова Б.Б., Суворова О.А., Каширская Н.Ю., Авдеев С.Н.Merzhoeva Z.M., Levina I.A., Lavginova B.B., Suvorova O.A., Kashirskaya N.Y., Avdeev S.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/5040

Тройная CFTR­модулирующая терапия ивакафтор + тезакафтор + элексакафтор / ивакафтор (ИТЭ) существенно изменила течение и прогноз муковисцидоза (МВ). Расширение применения биоэквивалентного генерического препарата (ГП) Трилекса® определяет необходимость обобщения данных о его клинической эффективности и безопасности, в т. ч. при переходе с оригинального препарата (ОП) Трикафта®.

Целями исследования являлись обобщение и систематизация отечественных и международных данных об эффективности и безопасности тройной CFTR­модулирующей терапии ИТЭ, включая опыт применения ОП и ГП, у детей и взрослых с МВ.

Результаты

Результаты. Тройная комбинация ИТЭ является наиболее изученным и эффективным из доступных методов патогенетической терапии МВ. По данным рандомизированных клинических исследований и наблюдательных программ при назначении ОП обеспечиваются прирост объема форсированного выдоха на 1­ю секунду (ОФВ1), выраженное снижение хлоридов пота, улучшение нутритивного статуса и значимое снижение частоты легочных обострений. ГП обладает подтвержденной биоэквивалентностью. По результатам российских многоцентровых и региональных исследований убедительно продемонстрировано, что переход с ОП на ГП сопровождается сохранением ОФВ 1, форсированной жизненной емкости легких (ФЖЕЛ), нутритивного статуса и суррогатных маркеров CFTR­функции при сохранении благоприятного профиля безопасности. Накопленный клинический опыт включает первый задокументированный случай успешного завершения беременности на фоне терапии ИТЭ. Дополнительно рассмотрены данные исследований по эффективности и безопасности комбинации дейтивакафтор + тезакафтор + ванзакафтор как новой опции тройной CFTR­модулирующей терапии.

Заключение

Заключение. Совокупность данных международных рандомизированных клинических исследований, регистровых исследований и российского регионального опыта свидетельствует о клинической сопоставимости ГП и ОП при переходе в рамках одного международного непатентованного наименования. В условиях расширения охвата таргетной терапией применение биоэквивалентного генерического аналога является обоснованной стратегией повышения доступности патогенетического лечения.

Triple CFTR modulator therapy with ivacaftor + tezacaftor + elexacaftor and ivacaftor (ITE) has substantially changed the clinical course and prognosis of cystic fibrosis. The expanding use of the bioequivalent generic Trilexa® highlights the need to summarize evidence on its clinical efficacy and safety, including outcomes after switching from the originator Trikafta®.

The aim of this study was to summarize and systematize national and international data on the efficacy and safety of triple CFTR modulator therapy with ITE, including the experience of using the original drug and the generic drug, in children and adults with cystic fibrosis.

Results

Results. The triple ITE combination is the most widely studied and the most effective of the available methods of pathogenetic therapy for cystic fibrosis. In randomized clinical trials and observational programs, the originator provides an increase in FEV 1, a marked reduction in sweat chloride concentration, improvement in nutritional status, and a significant decrease in the rate of pulmonary exacerbations. Bioequivalence of the generic drug has been confirmed. Russian multicenter and regional studies convincingly show that switching from the originator to bioequivalent generic is accompanied by maintenance of FEV1, FVC, nutritional status, and surrogate markers of CFTR function, while preserving a favorable safety profile. The accumulated clinical experience includes the first documented case of a successfully completed pregnancy and delivery under treatment with a generic ITE regimen. Data from studies evaluating the efficacy and safety of deutivacaftor + tezacaftor + vanzacaftor as a new option for triple CFTR modulator therapy were additionally reviewed.

Conclusion

Conclusion. The body of evidence from international randomized clinical trials, registry studies, and Russian regional experience indicates clinical comparability between the originator and bioequivalent generic when switching from one to another. The use of a bioequivalent generic is a justified strategy to improve the availability of pathogenetic treatment with the goal to expand access to targeted therapy.

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