Lymphangioleiomyomatosis, sporadic and associated with tuberous sclerosis: a pulmonologist’s point of view
https://doi.org/10.18093/0869-0189-2025-35-3-403-411
Abstract
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.
Keywords
sporadic lymphangioleiomyomatosis,
tuberous sclerosis,
pulmonary cysts,
multifocal micronodular pneumocyte hyperplasia,
angiomyolipoma,
lymphangioleiomyoma
Supporting agencies: The authors declare that no external funding was provided for the study or publication
About the Authors
M. A. Makarova
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 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 Federation
Competing Interests:
Russian Federation
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
Competing Interests:
No conflict of interest is declared by the authors
A. S. Belevskiy
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
Russian Federation
Competing Interests:
Russian Federation
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
Competing Interests:
No conflict of interest is declared by the authors
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Makarova M.A., Belevskiy A.S. Lymphangioleiomyomatosis, sporadic and associated with tuberous sclerosis: a pulmonologist’s point of view. PULMONOLOGIYA. 2025;35(3):403-411. (In Russ.) https://doi.org/10.18093/0869-0189-2025-35-3-403-411
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