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Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management

Pulmonary sporadic lymphangioleiomyomatosis (LAM) is a female-dominant disease associated with smooth muscle cell proliferation, which results in cystic lung disease presenting commonly with dyspnea and pneumothorax. This article aims to present a patient with the common clinical features and compli...

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Autores principales: Kania, Brooke E, Jain, Swapna, West, Brittanie, Courtney, Suzanne W
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331909/
https://www.ncbi.nlm.nih.gov/pubmed/32637268
http://dx.doi.org/10.7759/cureus.8386
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author Kania, Brooke E
Jain, Swapna
West, Brittanie
Courtney, Suzanne W
author_facet Kania, Brooke E
Jain, Swapna
West, Brittanie
Courtney, Suzanne W
author_sort Kania, Brooke E
collection PubMed
description Pulmonary sporadic lymphangioleiomyomatosis (LAM) is a female-dominant disease associated with smooth muscle cell proliferation, which results in cystic lung disease presenting commonly with dyspnea and pneumothorax. This article aims to present a patient with the common clinical features and complications of LAM so as to aid in the efficient diagnosis and treatment of future patients. Limited options in the management of LAM make early diagnosis key, as management focuses on supportive care to slow the progressive decline of pulmonary function. Workup includes a diagnosis of exclusion with specific antibodies or titers such as anti-Sjögren's syndrome type A (anti-SSA) antibodies, anti-Sjögren's syndrome type B (anti-SSB) antibodies, angiotensin-converting enzyme (ACE) levels, alpha-1-antitrypsin levels, and vascular endothelial growth factor (VEGF) antibodies with definitive diagnosis limited to tissue confirmation. Here, we discuss a 39-year-old female with dyspnea and spontaneous pneumothorax, who was subsequently diagnosed with LAM during her hospitalization and managed outpatient with sirolimus therapy.
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spelling pubmed-73319092020-07-06 Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management Kania, Brooke E Jain, Swapna West, Brittanie Courtney, Suzanne W Cureus Cardiac/Thoracic/Vascular Surgery Pulmonary sporadic lymphangioleiomyomatosis (LAM) is a female-dominant disease associated with smooth muscle cell proliferation, which results in cystic lung disease presenting commonly with dyspnea and pneumothorax. This article aims to present a patient with the common clinical features and complications of LAM so as to aid in the efficient diagnosis and treatment of future patients. Limited options in the management of LAM make early diagnosis key, as management focuses on supportive care to slow the progressive decline of pulmonary function. Workup includes a diagnosis of exclusion with specific antibodies or titers such as anti-Sjögren's syndrome type A (anti-SSA) antibodies, anti-Sjögren's syndrome type B (anti-SSB) antibodies, angiotensin-converting enzyme (ACE) levels, alpha-1-antitrypsin levels, and vascular endothelial growth factor (VEGF) antibodies with definitive diagnosis limited to tissue confirmation. Here, we discuss a 39-year-old female with dyspnea and spontaneous pneumothorax, who was subsequently diagnosed with LAM during her hospitalization and managed outpatient with sirolimus therapy. Cureus 2020-06-01 /pmc/articles/PMC7331909/ /pubmed/32637268 http://dx.doi.org/10.7759/cureus.8386 Text en Copyright © 2020, Kania et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Cardiac/Thoracic/Vascular Surgery
Kania, Brooke E
Jain, Swapna
West, Brittanie
Courtney, Suzanne W
Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management
title Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management
title_full Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management
title_fullStr Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management
title_full_unstemmed Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management
title_short Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management
title_sort lymphangioleiomyomatosis: a case report and review of clinical features and management
topic Cardiac/Thoracic/Vascular Surgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331909/
https://www.ncbi.nlm.nih.gov/pubmed/32637268
http://dx.doi.org/10.7759/cureus.8386
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