Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis
ABSTRACT Lymphangioleiomyomatosis (LAM) is a rare disease, characterized as a low-grade neoplasm with metastatic potential that mainly affects women of reproductive age, in which there is proliferation of atypical smooth muscle cells (LAM cells) and formation of diffuse pulmonary cysts. It can occur...
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Sociedade Brasileira de Pneumologia e Tisiologia
2025-02-01
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| Series: | Jornal Brasileiro de Pneumologia |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132025000100700&lng=en&tlng=en |
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| author | Bruno Guedes Baldi Paulo Henrique Ramos Feitosa Adalberto Sperb Rubin Alexandre Franco Amaral Carolina Salim Gonçalves Freitas Cláudia Henrique da Costa Eliane Viana Mancuzo Ellen Caroline Toledo do Nascimento Mariana Sponholz Araujo Marcelo Jorge Jacó Rocha Martina Rodrigues de Oliveira Tatiana Senna Galvão Pedro Paulo Teixeira e Silva Torres Carlos Roberto Ribeiro Carvalho |
| author_facet | Bruno Guedes Baldi Paulo Henrique Ramos Feitosa Adalberto Sperb Rubin Alexandre Franco Amaral Carolina Salim Gonçalves Freitas Cláudia Henrique da Costa Eliane Viana Mancuzo Ellen Caroline Toledo do Nascimento Mariana Sponholz Araujo Marcelo Jorge Jacó Rocha Martina Rodrigues de Oliveira Tatiana Senna Galvão Pedro Paulo Teixeira e Silva Torres Carlos Roberto Ribeiro Carvalho |
| author_sort | Bruno Guedes Baldi |
| collection | DOAJ |
| description | ABSTRACT Lymphangioleiomyomatosis (LAM) is a rare disease, characterized as a low-grade neoplasm with metastatic potential that mainly affects women of reproductive age, in which there is proliferation of atypical smooth muscle cells (LAM cells) and formation of diffuse pulmonary cysts. It can occur in a sporadic form or in combination with tuberous sclerosis complex. In recent decades, a number of advances have been made in the understanding of the pathophysiology and management of LAM, leading to improvements in its prognosis: identification of the main genetic aspects and the role of the mechanistic target of rapamycin (mTOR) pathway; relationship with hormonal factors, mainly estrogen; characterization of pulmonary and extrapulmonary manifestations in imaging studies; identification and importance in the diagnosis of VEGF-D; a systematic diagnostic approach, often without the need for lung biopsy; use of and indications for the use of mTOR inhibitors, mainly sirolimus, for pulmonary and extrapulmonary manifestations; pulmonary rehabilitation and the management of complications such as pneumothorax and chylothorax; and the role of and indications for lung transplantation. To date, no Brazilian recommendations for a comprehensive approach to the disease have been published. This document is the result of a non-systematic review of the literature, carried out by 12 pulmonologists, a radiologist, and a pathologist, which aims to provide an update of the most important topics related to LAM, mainly to its diagnosis, treatment, and follow-up, including practical and multidisciplinary aspects of its management. |
| format | Article |
| id | doaj-art-6815c9d588a646aa8b973fc76a8504a7 |
| institution | Kabale University |
| issn | 1806-3756 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Sociedade Brasileira de Pneumologia e Tisiologia |
| record_format | Article |
| series | Jornal Brasileiro de Pneumologia |
| spelling | doaj-art-6815c9d588a646aa8b973fc76a8504a72025-02-11T07:43:21ZengSociedade Brasileira de Pneumologia e TisiologiaJornal Brasileiro de Pneumologia1806-37562025-02-0151110.36416/1806-3756/e20240378Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosisBruno Guedes Baldihttps://orcid.org/0000-0002-9609-5117Paulo Henrique Ramos Feitosahttps://orcid.org/0000-0002-9029-7033Adalberto Sperb Rubinhttps://orcid.org/0000-0002-4591-1444Alexandre Franco Amaralhttps://orcid.org/0000-0001-6522-568XCarolina Salim Gonçalves Freitashttps://orcid.org/0000-0002-9580-1454Cláudia Henrique da Costahttps://orcid.org/0000-0001-6785-0753Eliane Viana Mancuzohttps://orcid.org/0000-0003-3891-875XEllen Caroline Toledo do Nascimentohttps://orcid.org/0000-0002-2981-1419Mariana Sponholz Araujohttps://orcid.org/0000-0001-9470-9333Marcelo Jorge Jacó Rochahttps://orcid.org/0009-0003-5441-9990Martina Rodrigues de Oliveirahttps://orcid.org/0000-0002-4990-6394Tatiana Senna Galvãohttps://orcid.org/0000-0002-3038-7715Pedro Paulo Teixeira e Silva Torreshttps://orcid.org/0000-0002-8571-5667Carlos Roberto Ribeiro Carvalhohttps://orcid.org/0000-0002-1618-8509ABSTRACT Lymphangioleiomyomatosis (LAM) is a rare disease, characterized as a low-grade neoplasm with metastatic potential that mainly affects women of reproductive age, in which there is proliferation of atypical smooth muscle cells (LAM cells) and formation of diffuse pulmonary cysts. It can occur in a sporadic form or in combination with tuberous sclerosis complex. In recent decades, a number of advances have been made in the understanding of the pathophysiology and management of LAM, leading to improvements in its prognosis: identification of the main genetic aspects and the role of the mechanistic target of rapamycin (mTOR) pathway; relationship with hormonal factors, mainly estrogen; characterization of pulmonary and extrapulmonary manifestations in imaging studies; identification and importance in the diagnosis of VEGF-D; a systematic diagnostic approach, often without the need for lung biopsy; use of and indications for the use of mTOR inhibitors, mainly sirolimus, for pulmonary and extrapulmonary manifestations; pulmonary rehabilitation and the management of complications such as pneumothorax and chylothorax; and the role of and indications for lung transplantation. To date, no Brazilian recommendations for a comprehensive approach to the disease have been published. This document is the result of a non-systematic review of the literature, carried out by 12 pulmonologists, a radiologist, and a pathologist, which aims to provide an update of the most important topics related to LAM, mainly to its diagnosis, treatment, and follow-up, including practical and multidisciplinary aspects of its management.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132025000100700&lng=en&tlng=enLymphangioleiomyomatosis/diagnosisLymphangioleiomyomatosis/prevention & controlLymphangioleiomyomatosis/pathophysiologyLymphangioleiomyomatosis/drug treatmentLymphangioleiomyomatosis/therapyClinical practice guide |
| spellingShingle | Bruno Guedes Baldi Paulo Henrique Ramos Feitosa Adalberto Sperb Rubin Alexandre Franco Amaral Carolina Salim Gonçalves Freitas Cláudia Henrique da Costa Eliane Viana Mancuzo Ellen Caroline Toledo do Nascimento Mariana Sponholz Araujo Marcelo Jorge Jacó Rocha Martina Rodrigues de Oliveira Tatiana Senna Galvão Pedro Paulo Teixeira e Silva Torres Carlos Roberto Ribeiro Carvalho Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis Jornal Brasileiro de Pneumologia Lymphangioleiomyomatosis/diagnosis Lymphangioleiomyomatosis/prevention & control Lymphangioleiomyomatosis/pathophysiology Lymphangioleiomyomatosis/drug treatment Lymphangioleiomyomatosis/therapy Clinical practice guide |
| title | Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis |
| title_full | Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis |
| title_fullStr | Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis |
| title_full_unstemmed | Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis |
| title_short | Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis |
| title_sort | brazilian thoracic association recommendations for the management of lymphangioleiomyomatosis |
| topic | Lymphangioleiomyomatosis/diagnosis Lymphangioleiomyomatosis/prevention & control Lymphangioleiomyomatosis/pathophysiology Lymphangioleiomyomatosis/drug treatment Lymphangioleiomyomatosis/therapy Clinical practice guide |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132025000100700&lng=en&tlng=en |
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