pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2025-35-3-403-411pulmo-4605Research ArticleОБЗОРЫREVIEWЛимфангиолейомиоматоз спорадический и ассоциированный с туберозным склерозом: взгляд пульмонологаLymphangioleiomyomatosis, sporadic and associated with tuberous sclerosis: a pulmonologist’s point of viewhttps://orcid.org/0000-0003-4913-087XМакароваМ. А.MakarovaM. A.

Макарова Марина Алексеевна – к. м. н., доцент кафедры пульмонологии Института непрерывного образования и профессионального развития Российский национальный исследовательский медицинский университет имени Н.И. Пирогова; научный сотрудник лаборатории интенсивной терапии и дыхательной недостаточности Научно-исследовательский институт пульмонологии; врач-пульмонолог отделения респираторной медицины Городская клиническая больница имени С.С. Юдина Департамента здравоохранения города Москвы.

117997, Москва, ул. Островитянова, 1; 115682, Москва, Ореховый бульвар, 28; 115446, Москва, Коломенский проезд, 4, тел.: (499) 780-08-43; Author ID: 766549

Marina A. Makarova - Candidate of Medicine, Associate Professor, Department of Pulmonology, Institute of Continuous Education and Professional Development, N.I. Pirogov Russian National Research Medical University; Researcher, Laboratory of Intensive Care and Respiratory Failure, Pulmonology Scientific Research Institute; Pulmonologist, Department of Respiratory Medicine, Moscow State Budgetary Healthcare Institution “Moscow City Hospital named after S.S. Yudin, Moscow Healthcare Department”.

Ul. Ostrovityanova 1, Moscow, 117997; Orekhovyy bul’var 28, Moscow, 115682; Kolomenskiy pr. 4, Moscow, 115446; tel.: (499) 780-08-43; Author ID: 766549

mma123@list.ruhttps://orcid.org/0000-0001-6050-724XБелевскийА. С.BelevskiyA. S.

Белевский Андрей Станиславович – д. м. н., профессор, заведующий кафедрой пульмонологии Института непрерывного образования и профессионального развития.

117997, Москва, ул. Островитянова, 1; тел.: (495) 963-24-67

Andrey S. Belevskiy - Doctor of Medicine, Professor; Head of the Pulmonology Department, Institute of Continuous Education and Professional Development.

Ul. Ostrovityanova 1, Moscow, 117997; tel.: (495) 963-24-67

pulmobas@yandex.ruФедеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской Федерации; Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства России; Государственное бюджетное учреждение здравоохранения города Москвы «Городская клиническая больница имени С.С. Юдина Департамента здравоохранения города Москвы»РоссияFederal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation; Federal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation; Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation; Moscow State Budgetary Healthcare Institution “Moscow City Hospital named after S.S.Yudin, Moscow Healthcare Department”Russian FederationФедеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияFederal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian FederationRussian Federation202525062025353403411Copyright © Макарова М.А., Белевский А.С., 20252025Макарова М.А., Белевский А.С.Makarova M.A., Belevskiy A.S.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/4605

Лимфангиолейомиоматоз (ЛАМ) – редкое заболевание с преимущественным диффузным кистозным поражением легких у женщин. В настоящее время при широкой доступности компьютерной томографии (КТ) количество пациентов с ЛАМ значительно увеличивается. Все это актуализирует важность и значимость знаний о данной орфанной болезни. В клинической практике ЛАМ встречается в виде самостоятельного заболевания – спорадический ЛАМ или как большой признак туберозного склероза (ТС) – ЛАМ, ассоциированный с ТС. Целью исследования явился обзор основных общих и разных проявлений обоих вариантов ЛАМ.

Заключение. Наряду с наличием общих клинических, рентгенологических и морфологических черт оба варианта ЛАМ имеют и существенные различия. В статье подробно обсуждаются основные особенности разных форм ЛАМ.

Lymphangioleiomyomatosis (LAM) is a rare disease with a predominant diffuse cystic lesion of the lungs in women. Currently, with the widespread availability of computed tomography (CT), the number of patients with LAM is increasing significantly. All this highlights the importance and significance of knowledge about this orphan disease. In clinical practice, LAM occurs either as an independent disease, sporadic LAM, or as LAM associated with tuberous sclerosis complex (TSC – LAM), a major sign of tuberous sclerosis. The aim was to present an overview of the main common and different manifestations of both variants of LAM.

Conclusion. Despite the common clinical, radiological and morphological features, both LAM variants have significant differences. The article discusses in detail the main features of different forms of LAMs.

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