pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2025-35-2-241-253pulmo-4680Research ArticleОБЗОРREVIEWОсобенности течения муковисцидоза у взрослых больных, инфицированных микроорганизмами Burkholderia cepacia complexThe course of cystic fibrosis in adult patients with Burkholderia cepacia complex infectionhttps://orcid.org/0000-0002-8260-7126АфанасьеваМ. В.AfanasevaM. V.

Мария Валерьевна Афанасьева, научный сотрудник

лаборатория муковисцидоза

115682; Ореховый бульвар, 28, стр. 10; Москва

тел.: (926) 139-54-66

Maria V. Afanaseva, Researcher

Cystic Fibrosis Department

115682; Orekhovyy bul’var 28, build. 10; Moscow

tel.: (926) 139-54-66

usa-mariya@yandex.ruhttps://orcid.org/0000-0001-9642-0947КрасовскийС. А.KrasovskiyS. A.

Станислав Александрович Красовский, к. м. н., старший научный сотрудник, исполняющий обязанности заведующего лабораторией, ведущий научный сотрудник

лаборатория муковисцидоза; научно-клинический отдел

115682; Ореховый бульвар, 28, стр. 10; 115522; ул. Москворечье, 1; Москва

тел.: (495) 111-03-03

Author ID: 688178

Stanislav A. Krasovskiy, Candidate of Medicine, Senior Researcher, Acting Head of the Laboratory, Leading Researcher

Cystic Fibrosis Laboratory; Scientific and Clinical Department

115682; Orekhovyy bul’var 28, build. 10; 115522; ul. Moskvorechye 1; Moscow

tel.: (495) 111-03-03

Author ID: 688178

sa_krasovsky@mail.ruhttps://orcid.org/0000-0002-5356-9415АмелинаЕ. Л.AmelinaE. L.

Елена Львовна Амелина,  к. м. н., заведующая лабораторией

лаборатория муковисцидоза

115682; Ореховый бульвар, 28, стр. 10; Москва

тел.: (495) 410-67-00

Author ID: 7003985681

Elena L. Amelina, Candidate of Medicine, Head of the Laboratory

Cystic Fibrosis Laboratory

115682; Orekhovyy bul’var 28, build. 10; Moscow

tel: (495) 410-67-00

Author ID: 7003985681

eamelina@mail.ruФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства РоссииРоссияFederal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian FederationRussian FederationФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства России; Федеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н.П.Бочкова» Министерства науки и высшего образования Российской ФедерацииРоссияFederal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation; Federal State Budgetary Scientific Institution “Research Centre for Medical Genetics”, Ministry of Science and Higher Education of the Russian FederationRussian Federation202518042025352241253Copyright © Афанасьева М.В., Красовский С.А., Амелина Е.Л., 20252025Афанасьева М.В., Красовский С.А., Амелина Е.Л.Afanaseva M.V., Krasovskiy S.A., Amelina 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://journal.pulmonology.ru/pulm/article/view/4680

   Муковисцидоз (МВ) – полиорганное заболевание, однако его течение и прогноз в большинстве случаев определяются патологией бронхолегочной системы. Одним из наиболее опасных возбудителей для пациентов с МВ является Burkholderia cepacia complex (Bcc) – грамотрицательные бактерии, характеризуемые высокой трансмиссивностью и патогенностью, а также природной резистентностью к широкому спектру антимикробных препаратов и быстрым приобретением устойчивости к новым антибактериальным препаратам. При этом наблюдаются серьезные ограничения терапевтических возможностей эрадикации и лечения Всс, прогрессивно снижается функция легких и значительно лимитируется продолжительность жизни больных. Для Bсс также описан «сепациа-синдром» – быстро нарастающая дыхательная недостаточность, обусловленная некротизирующей пневмонией. Россия относится к странам с относительно высокой распространенностью Всс – 5,5 %.

   Целью обзора явился анализ литературных источников, по данным которых оцениваются распространенность Всс у взрослых пациентов с МВ в Российской Федерации, влияние хронической инфекции Всс на течение МВ и выживаемость пациентов, в т. ч. после трансплантации легких, возможности ее эрадикации и лечения.

   У российских больных чаще встречается Burkholderia cenocepacia ST709 – эпидемический вид, вызывающий типичную госпитальную инфекцию, источником которой являются пациенты с МВ. Эффективных схем эрадикации и лечения Всс не разработано. При таргетной терапии модулятором ивакафтор / тезакафтор / элексакафтор + ивакафтор степень обсемененности Всс в мокроте снижается, но не приводит к ее эрадикации. Трансплантация легких у больных с Всс проходит с многочисленными осложнениями, может потребоваться длительная реабилитация в посттрансплантационном периоде. Инфицирование госпитальными видами Всс связано с более тяжелым течением заболевания и низкой выживаемостью, а инфицирование менее эпидемическими видами Всс, вероятно, определяет лучший прогноз для пациентов с МВ. Роль инфицирования больных «дикими» видами Всс не определена и требует дальнейшего изучения.

   Заключение. Таким образом, вопросы профилактики перекрестного инфицирования пациентов с Всс остаются актуальными.

   Cystic fibrosis (CF) is a multi-organ disease, but its course and prognosis in most cases are determined by the pathology of the bronchopulmonary system. One of the most harmful pathogens for patients with CF is Burkholderia cepacia complex (Bcc). These are gram-negative bacteria characterized by high transmissibility and pathogenicity, as well as natural resistance to a wide range of antimicrobial drugs and rapid acquisition of resistance to new antibiotics. This seriously limits the therapeutic possibilities of eradication and treatment of Bcc, progressively reduces the lung function, and significantly limits the life expectancy of patients. A “cepacia syndrome” has also been described for Bcc – a rapidly increasing respiratory failure due to necrotizing pneumonia. Russia belongs to the countries with a relatively high Bcc prevalence of 5.5 %.

   The aim of the review was to analyze the literature data on the prevalence of Bcc in adult patients with CF in the Russian Federation, the impact of chronic Bcc infection on the course of CF and survival, including after lung transplantation, and the possibility of its eradication and treatment. Burkholderia cenocepacia ST709, an epidemic species that causes a typical hospital infection, the source of which is patients with CF, is more common in Russian patients. No effective schemes for the eradication and treatment of Bcc have been developed. Targeted therapy with ivacaftor + thesacaftor + elecsacaftor and ivacaftor modulator reduce the degree of Bcc contamination in sputum, but do not lead to its eradication. Lung transplantation in patients with Bcc proceeds with a large number of complications and may require long-term rehabilitation in the post-transplant period. Infection with hospital-acquired species of Bcc is associated with a more severe course of the disease and low survival, and infection with less epidemic species of Bcc probably determines the best prognosis for patients with CF. The role of infection of patients with “wild” species of Bcc has not been determined and requires further study.

   Conclusion. Prevention of cross-infection in patients with Всс remains a pressing issue.

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