Lymphangioleiomyomatosis: Current and Future

Overview

Journal J Thorac Dis

Publisher AME Publishing Company

Specialty Pulmonary Medicine

Date 2013 Feb 2

PMID 23372952

Citations 12

Authors

Maria Mavroudi

Paul Zarogoulidis

Nikolaos Katsikogiannis

Kosmas Tsakiridis

Haidong Huang

Antonios Sakkas

Anastasios Kallianos

Aggeliki Rapti

Eirini Sarika

Ilias Karapantzos

Konstantinos Zarogoulidis

Affiliations

Soon will be listed here.

Abstract

Lymphangioleiomyomatosis is a rare slowly progressive lung disease that affects almost exclusively young women of reproductive age. It occurs sporadically or in association with Tuberous Sclerosis Complex. LAM is characterized by cystic remodeling of the lung parenchyma, due to proliferation of abnormal smooth muscle-like LAM cells and presence of extra pulmonary manifestations such as lymphadenopathy, angiomyolipomas and abdominal lymphangioleiomyomas. The most common clinical manifestations are progressive dyspnea on exertion, pneumothorax and chylous effusions. Currently there is no curative treatment for the disease, but the ongoing study of the genetic and molecular pathways implicated in the pathogenesis of the disease could lead to targeted therapy.

Citing Articles

Case report: If it is not asthma-think of lymphangioleiomyomatosis in younger female patients.

Kirkeby M, Bendstrup E, Rose H Front Med (Lausanne). 2024; 11:1328471.

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Benign metastasizing uterine leiomyoma with lymphatic and pulmonary metastases: a case report and literature review.

Tong T, Fan Q, Wang Y, Li Y BMC Womens Health. 2023; 23(1):154.

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Pulmonary lymphangioleiomyomatosis: a case report.

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Effects of Sirolimus on Lung function in patients with Lymphangioleiomyomatosis.

Aghaeimeybodi F, Najafizadeh K, Razavi-Ratki S, Namiranian N Caspian J Intern Med. 2019; 10(1):7-10.

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Lymphangioleiomyomatosis: a case report and review of diagnosis and treatment.

Liu Y, Guo Z, Zhao C, Li X, Liu H, Chen J Onco Targets Ther. 2018; 11:5339-5347.

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