Consilium MedicumConsilium Medicum2075-17532542-2170Consilium Medicum10806010.26442/20751753.2022.3.201504Research ArticleLymphangioleiomyomatosis of the lungs: features of the clinical picture, diagnosis and treatment. Case reportIgnatovaGalina L.<p>D. Sci. (Med.), Prof.</p>iglign@mail.ruhttps://orcid.org/0000-0002-0877-6554BlinovaElena V.<p>Cand. Sci. (Med.)</p>blinel@mail.ruhttps://orcid.org/0000-0003-2507-5941Bel'snerMariia S.<p>Cand. Sci. (Med.)</p>mariika_04@mail.ruSouth Ural State Medical University190320222431661691905202219052022Copyright © 2022, Consilium Medicum2022<p>The article highlights the main issues of etiology, pathogenesis, clinical picture, diagnosis and treatment of patients with lymphangioleiomyomatosis. The clinical observation presents the features of the condition, the rate of progression, diagnostic approaches and management programs for a patient with a rare cystic lung pathology.</p>lymphangioleiomyomatosismTOR inhibitorsclinical caseлимфангиолейомиоматозингибиторы mTORклинический случай[Meraj R, Wikenheiser-Brokamp KA, Young LR, McCormack FX. Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 2012;33(5):486-947. DOI:10.1055/s-0032-1325159][Ando K, Tobino K, Kurihara M, et al. Quantitative CT analysis of small pulmonary vessels in lymphangioleiomyomatosis. Eur J Radiol. 2012;81(12):3925-30. DOI:10.1016/j.ejrad.2012.05.033][Seibert D, Hong CH, Takeuchi F, et al. Recognition of tuberous sclerosis in adult women: delauyed presentation with life-treatening consequences. Ann Intern Med. 2011;154(12):806-13,W-294. DOI:10.7326/0003-4819-154-12-201106210-00008][Zhang X, Travis WD. Pulmonary lymphangioleiomyomatosis. Arch Pathol Lab Med. 2010;134(12):1823-8. DOI:10.5858/2009-0576-RS.1][Johnson SR, Cordier JF, Lazor R, et al. Review Panel of the ERS LAM Task Force. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010;35(26):14-26. DOI:10.1183/09031936.00076209][Harari S, Torre O, Moss J. Lymphangioleiomyomatosis: what do we know and what are we looking for? Eur Respir J. 2011;20(119):34-44. DOI:10.1183/09059180.00011010][Cordier JF, Johnson SR. Multiple cystic lung diseases. Eur Respir Monogr. 2011;54:46-83.][McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011;364:1595-606. DOI:10.1056/NEJMoa1100391][Kpodonu J, Massad MG, Chaer RA, et al. The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis. J Heart Lung Transplant. 2005; 24(9):1247-53. DOI:10.1016/j.healun.2004.09.013]