Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study

Abstract

Background Spontaneous pneumothorax has a high incidence and high rate of recurrence in patients with lymphangioleiomyomatosis (LAM). The risk factors for pneumothorax and the effects of sirolimus on pneumothorax in patients with LAM are unknown. In our study, multivariate logistic regression was applied to a cross-sectional cohort to investigate factors associated with pneumothorax in LAM patients. Kaplan–Meier analysis was applied in the historical prospective self-controlled study to determine whether sirolimus reduces the risk of pneumothorax recurrence in patients with LAM. Results Of the 399 patients registered with LAM-CHINA at our center between May 10, 2017 and August 31, 2020, 142 had a history of pneumothorax at registration. High CT grade and age at presentation ≤ 35 years were associated with a higher risk of pneumothorax in patients with LAM. Postmenopausal status was correlated with a lower risk of pneumothorax. In the historical prospective self-controlled study, the 5-year probability of pneumothorax recurrence was 80% lower in the sirolimus group than in the control group (hazard ratio for pneumothorax recurrence, 0.20; 95% CI, 0.14 to 0.30, P < 0.001 by log-rank test). Conclusion Sirolimus reduced the risk of pneumothorax recurrence in LAM patients.

Intraoperative mechanical ventilation and incidence of pneumothorax in lymphangioleiomyomatosis

“…Higher PaO 2 and SpO 2 on room air may also convey a safer message for mechanical ventilation if patients are unsuitable for pulmonary function testing. Consistent with the potential protective effect of mTOR inhibitors in LAM patients [10] therapy in our study did not experience postoperative PTX even under mechanical ventilation.…”

Recurrent Pneumothorax Caused by an Unexpected Lymphangioleiomyomatosis: A Case Report

“…Due to the high recurrence rate of pneumothorax in patients with LAM, pleurodesis is suggested for patients after the first episode of pneumothorax [1]. A recent study showed that sirolimus, a mammalian target of rapamycin inhibitor, significantly reduced the risk of pneumothorax recurrence in patients with LAM [6]. In our case, we only performed pulmonary wedge resection without pleurodesis because we did not consider the possibility of LAM.…”

“…In patients with LAM, the pulmonary function test (PFT) may be significant if there is a decrease in the forced expiratory volume in 1 second (FEV1) and diffusion capacity for carbon monoxide . Patients with a history of pneumothorax have worse pulmonary function than patients without a history of pneumothorax [6]. Steagall et al [5] reported that the rate of decline in FEV1 was higher in patients with pneumothorax in the early stage of LAM than in those without pneumothorax in the early stage of LAM.…”

“…Repeated pneumothorax may cause acute exacerbation of LAM [6]. Due to the high recurrence rate of pneumothorax in patients with LAM, pleurodesis is suggested for patients after the first episode of pneumothorax [1].…”

A Group Contribution Analogy as Selection Criteria for Nutrients Fermentation Media in the Production of Tacrolimus, Sirolimus and Ascomycin

“…Sirolimus (Fig. 2), empirical formula C 51 H 79 NO 13, also known as rapamycin with molar mass 914.17 g/mol and sold under the brand name Rapamune is a macrolide compound that is used to coat coronary stents, prevent organ transplant rejection, treat a rare lung disease called lymphangioleiomyomatosis [9], and treat perivascular epithelioid cell tumor (PEComa) [10]. It has immunosuppressant functions in humans and is especially useful in preventing the rejection of kidney transplants [11].Sirolimus was initially developed as an antifungal agent [12].…”