pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2023-33-6-820-831pulmo-4170Research ArticleОБЗОРЫREVIEWЛегочное кровотечение при муковисцидозе: обзор актуальных данныхPulmonary hemorrhage in cystic fibrosis: a review of current datahttps://orcid.org/0000-0001-7655-7911ФедотоваМ. М.FedotovaM. M.

Федотова Марина Михайловна – доцент кафедры факультетской педиатрии с курсом детских болезней лечебного факультета.

634050, Томск, Московский тракт, 2; тел.: (3822) 901-101 (доб. 1734)

Marina M. Fedotova - Associate Professor, Department of Faculty Pediatrics with a course of childhood diseases, Faculty of Medicine.

Moskovskiy trakt 2, Tomsk, 634050; tel.: (3822) 901-101 (доб. 1734)

fedotova.letter@gmail.comhttps://orcid.org/0000-0003-1321-8909ДоронинаА. А.DoroninaA. A.

Доронина Александра Артуровна – ординатор кафедры факультетской педиатрии с курсом детских болезней лечебного факультета.

634050, Томск, Московский тракт, 2; тел.: (3822) 90-11-01 (доб. 1734)

Aleksandra A. Doronina - Resident, Department of Faculty Pediatrics with a course of childhood diseases, Faculty of Medicine.

Moskovskiy trakt 2, Tomsk, 634050; tel.: (3822) 90-11-01 (add. 1734)

sasha_dorn98@mail.ruФедеральное государственное бюджетное образовательное учреждение высшего образования «Сибирский государственный медицинский университет» Министерства здравоохранения Российской ФедерацииРоссияFederal State Budgetary Educational Institution of Higher Education “Siberian State Medical University” of the Ministry of Health of the Russian FederationRussian Federation202313122023336820831Copyright © Федотова М.М., Доронина А.А., 20232023Федотова М.М., Доронина А.А.Fedotova M.M., Doronina A.A.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/4170

Легочное кровотечение (ЛК), распространенность которого достигает 9,1 %, является серьезным осложнением при муковисцидозе (МВ). По данным Регистра пациентов с МВ Российской Федерации (2020), распространенность ЛК составила 1,5 %, а среди пациентов старше 18 лет – 6,5 %. Несмотря на актуальность данной проблемы, в настоящее время систематизированная информация о ЛК при МВ отсутствует.

Целью работы явился обзор актуальных данных о патогенезе, факторах риска и подходах к лечению ЛК у пациентов с МВ. Поиск информации проводился по электронным базам данных еLibrary и PubMed.

Результаты

Результаты. По результатам анализа литературных источников обобщены и систематизированы накопленные научные данные в отношении ЛК при МВ. Установлено, что основой патогенеза ЛК является хроническое воспаление в стенке бронхов, которое сопровождается повышенной продукцией факторов ангиогенеза и приводит к образованию извилистых тонкостенных сосудов. Обострение воспалительного процесса в стенке бронхов и механические нагрузки способствуют эрозии и повреждению хрупких сосудов и становятся причиной развития ЛК. Основными факторами риска данного осложнения являются колонизация Pseudomonas aeruginosa, Staphylococcus aureus, наличие сахарного диабета, снижение объема форсированного выдоха в 1-ю секунду < 70 %. При комплексной терапии ЛК применяются антибактериальные и антифибринолитические препараты; также опубликованы схемы использования β-блокаторов, VII фактора свертывания крови. Данные об эффективности витамина К неоднозначны, однако указанный препарат также используется в составе комплексной терапии данного осложнения. При массивных ЛК (МЛК) рекомендуется временно прекратить ингаляции гипертонического раствора и кинезиотерапевтические приемы с положительным давлением на выдохе. При рецидивирующих МЛК выполняется эмболизация бронхиальных артерий, в исключительных случаях проводится резекция легкого.

Заключение

Заключение. Кровохарканье при МВ представляет собой сложную клиническую проблему, при решении которой требуется мультидисциплинарный подход. В настоящее время важные аспекты патогенеза ЛК остаются недостаточно изученными. Учитывая увеличение продолжительности жизни пациентов с МВ и связанное с возрастом повышение риска развития ЛК, актуальным является изучение различных патогенетических аспектов данного осложнения для последующей разработки эффективных и обоснованных алгоритмов лечения.

Pulmonary hemorrhage is a serious complication of cystic fibrosis (CF) with a prevalence up to 9.1%. According to the Registry of Patients with Cystic Fibrosis of the Russian Federation for 2020, the prevalence of pulmonary hemorrhage was 1.5% in the general population, and 6.5% in patients over 18 years of age. Despite the importance of this problem, there is no systematic information on pulmonary hemorrhage in CF.

Aim

Aim. The purpose of this study was to review current data on the pathogenesis, risk factors, and approaches to the treatment of pulmonary hemorrhage in patients with CF. Information was searched in еLibrary and PubMed databases.

Results

Results. During the literature review, we summarized and systematized the collected scientific data on pulmonary hemorrhage in CF. The pathogenesis of pulmonary hemorrhage is chronic inflammation in the bronchial wall accompanied by increased angiogenesis factors that promote neovascularization with development of many tortuous, thin-walled vessels. Exacerbation of bronchial inflammation and mechanical strain contribute to erosion and damage of fragile vessels and cause the bleeding. The main risk factors for pulmonary hemorrhage were colonization with Pseudomonas aeruginosa, Staphylococcus aureus, diabetes mellitus, and FEV1 < 70%. Complex therapy for pulmonary hemorrhage includes the use of antibiotics and antifibrinolytic agents. Some of the published regimens use β-blockers and blood coagulation factor VII. Data on the efficacy of vitamin K are unclear, but it is also used in the treatment of pulmonary hemorrhage. In cases of massive bleeding, temporarily discontinuation of inhalations of hypertonic solution and kinesiotherapy is recommended. In case of recurrent massive bleedings, bronchial artery embolization is performed and lung resection can be done in extreme cases.

Conclusion

Conclusion. Hemoptysis in CF is a complex clinical problem that requires a multidisciplinary approach. Currently, important aspects of the pathogenesis of pulmonary hemorrhage remain unclear. Given the increasing life expectancy of CF patients and the age-related risk of pulmonary hemorrhage, it is important to investigate the pathogenetic aspects of this complication for the subsequent development of effective and justified treatment algorithms.

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