Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case ser
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Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series Li Zhou, Ruoyun Ouyang*, Hong Luo, Siying Ren, Ping Chen, Yating Peng, Ting Liu and Guiqian Liu
Abstract Pneumothorax is one of the most common symptoms in patients with lymphangioleiomyomatosis (LAM). However, current management strategies for patients with LAM who present with recurrent pneumothorax remain inadequate. Here, we describe the successful prevention of recurrent pneumothorax by sirolimus treatment in five women with LAM. Before sirolimus treatment, all patients had received supplemental oxygen support, repeated chest tube drainage, or surgeries for management of the recurrent pneumothorax. Sirolimus treatment was initiated when the pneumothorax was completely resolved, and no patient developed pneumothorax during treatment. Moreover, they exhibited a significantly improved subjective quality of life, increased exercise capacity, and mild adverse effects such as mucositis, irregular menstruation, and delayed wound healing. On discontinuation of sirolimus or in the event that the plasma sirolimus level was markedly low, pneumothorax tended to relapse. The findings from these cases provide valuable insights that will aid in the improvement of treatment strategies for patients with LAM and recurrent pneumothorax. Keywords: Sirolimus, Lymphangioleiomyomatosis, Pneumothorax, Treatment strategy, Recurrence
Introduction Lymphangioleiomyomatosis (LAM) is a rare, progressive, cystic lung disease that mostly affects women of child-bearing age and is characterized by abnormal smooth muscle cell proliferation. LAM is associated with a series of clinical manifestations such as dyspnea, recurrent pneumothorax, hemoptysis, chylous effusion, renal angiomyolipoma (AML), retroperitoneal masses, and respiratory failure [1]. Pneumothorax is a common manifestation of LAM. Previous studies have demonstrated that approximately 66% of patients with LAM may exhibit pneumothorax; importantly, 70% of these patients may experience recurrent ipsilateral or contralateral pneumothoraces [2, 3]. However, management strategies for recurrent spontaneous pneumothorax in patients with LAM remain controversial and inadequate. Sirolimus, a common mammalian target of rapamycin (mTOR) inhibitor, is considered the first effective drug for patients with LAM. According to the guidelines * Correspondence: [email protected] Department of Pulmonary and Critical Care Medicine, The Second Xiangya Hospital, Central South University, 139 Renmin Road, Changsha, China
published in 2016 [4], sirolimus is recommended for the following types of patients with LAM: patients with moderately impaired lung function [a forced expiratory volume in 1 s (FEV1) of less than 70% predicted] or progressively declining lung function (decline rate for FEV1, over 90 ml/year) and patients with chylous effusion. However, to date, sirolimus has not been recommended for patients with LAM presenting with pneumot
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