Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis

Abstract Background Patients with lymphangioleiomyomatosis (LAM) frequently experience pneumothorax. Although sirolimus is the standard therapy for LAM, its effect on pneumothorax is controversial. Recently, total pleural covering (TPC) and modified TPC (mTPC) were introduced as surgical treatment o...

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Autores principales:	Teiko Sakurai, Toru Arai, Masaki Hirose, Kensuke Kojima, Tetsuki Sakamoto, Yoshinobu Matsuda, Chikatoshi Sugimoto, Hyung-Eun Yoon, Yoshikazu Inoue
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Lymphangioleiomyomatosis Pneumothorax Sirolimus Vascular endothelial growth factor D Total pleural covering Surgical pleural covering Medicine R
Acceso en línea:	https://doaj.org/article/fee158e7a1ac487b97531e02ca80e47b
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spelling	oai:doaj.org-article:fee158e7a1ac487b97531e02ca80e47b2021-11-08T10:56:57ZReduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis10.1186/s13023-021-02081-z1750-1172https://doaj.org/article/fee158e7a1ac487b97531e02ca80e47b2021-11-01T00:00:00Zhttps://doi.org/10.1186/s13023-021-02081-zhttps://doaj.org/toc/1750-1172Abstract Background Patients with lymphangioleiomyomatosis (LAM) frequently experience pneumothorax. Although sirolimus is the standard therapy for LAM, its effect on pneumothorax is controversial. Recently, total pleural covering (TPC) and modified TPC (mTPC) were introduced as surgical treatment options for pneumothorax for patients with LAM. However, the effect of sirolimus on the recurrence of pneumothorax in patients who underwent the treatments is still uncertain. We hypothesized that some clinical factors including sirolimus treatment could predict postoperative recurrence of pneumothorax. In order to clarify this hypothesis, we retrospectively analyzed the clinical data from 18 consecutive patients with LAM who underwent 24 surgical pleural covering of entire lung (SPC) as 17 TPC and 7 mTPC against pneumothoraces from surgical database between January 2005 and January 2019, and we determined the predictors of postoperative recurrence. Results Of the 24 surgeries of SPC, 14 surgeries (58.3%) had a history of two or more ipsilateral pneumothoraces, and 11 surgeries (45.8%) had a history of ipsilateral pleural procedures before SPC. Sixteen surgeries (66.6%) in 12 patients received treatment of sirolimus after SPC (sirolimus group). With a median follow-up time of 69.0 months after SPC, four surgeries (16.6%) in three patients had a postoperative recurrence, and the 5-year recurrence-free survival (RFS) after SPC was 82.9%. In patients with postoperative recurrence, serum level of vascular endothelial growth factors D was significantly higher than that in those with non-recurrence (3260.5 vs. 892.7 pg/mL, p = 0.02), and the rate of sirolimus treatment in the recurrence group was significantly lower than that in the no-recurrence group (0 vs. 80%, p = 0.006). The log-rank test showed that the RFS of the sirolimus group (sirolimus use after SPC) was significantly better than that of the non-sirolimus group (p = 0.001), and no significant difference was observed for other factors. Conclusion We first reported sirolimus might effectively suppress the recurrence of pneumothoraces in LAM patients who received SPC. Sirolimus induction after SPC (TPC or mTPC) might be a feasible option for frequent pneumothorax in LAM.Teiko SakuraiToru AraiMasaki HiroseKensuke KojimaTetsuki SakamotoYoshinobu MatsudaChikatoshi SugimotoHyung-Eun YoonYoshikazu InoueBMCarticleLymphangioleiomyomatosisPneumothoraxSirolimusVascular endothelial growth factor DTotal pleural coveringSurgical pleural coveringMedicineRENOrphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Lymphangioleiomyomatosis Pneumothorax Sirolimus Vascular endothelial growth factor D Total pleural covering Surgical pleural covering Medicine R
spellingShingle	Lymphangioleiomyomatosis Pneumothorax Sirolimus Vascular endothelial growth factor D Total pleural covering Surgical pleural covering Medicine R Teiko Sakurai Toru Arai Masaki Hirose Kensuke Kojima Tetsuki Sakamoto Yoshinobu Matsuda Chikatoshi Sugimoto Hyung-Eun Yoon Yoshikazu Inoue Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
description	Abstract Background Patients with lymphangioleiomyomatosis (LAM) frequently experience pneumothorax. Although sirolimus is the standard therapy for LAM, its effect on pneumothorax is controversial. Recently, total pleural covering (TPC) and modified TPC (mTPC) were introduced as surgical treatment options for pneumothorax for patients with LAM. However, the effect of sirolimus on the recurrence of pneumothorax in patients who underwent the treatments is still uncertain. We hypothesized that some clinical factors including sirolimus treatment could predict postoperative recurrence of pneumothorax. In order to clarify this hypothesis, we retrospectively analyzed the clinical data from 18 consecutive patients with LAM who underwent 24 surgical pleural covering of entire lung (SPC) as 17 TPC and 7 mTPC against pneumothoraces from surgical database between January 2005 and January 2019, and we determined the predictors of postoperative recurrence. Results Of the 24 surgeries of SPC, 14 surgeries (58.3%) had a history of two or more ipsilateral pneumothoraces, and 11 surgeries (45.8%) had a history of ipsilateral pleural procedures before SPC. Sixteen surgeries (66.6%) in 12 patients received treatment of sirolimus after SPC (sirolimus group). With a median follow-up time of 69.0 months after SPC, four surgeries (16.6%) in three patients had a postoperative recurrence, and the 5-year recurrence-free survival (RFS) after SPC was 82.9%. In patients with postoperative recurrence, serum level of vascular endothelial growth factors D was significantly higher than that in those with non-recurrence (3260.5 vs. 892.7 pg/mL, p = 0.02), and the rate of sirolimus treatment in the recurrence group was significantly lower than that in the no-recurrence group (0 vs. 80%, p = 0.006). The log-rank test showed that the RFS of the sirolimus group (sirolimus use after SPC) was significantly better than that of the non-sirolimus group (p = 0.001), and no significant difference was observed for other factors. Conclusion We first reported sirolimus might effectively suppress the recurrence of pneumothoraces in LAM patients who received SPC. Sirolimus induction after SPC (TPC or mTPC) might be a feasible option for frequent pneumothorax in LAM.
format	article
author	Teiko Sakurai Toru Arai Masaki Hirose Kensuke Kojima Tetsuki Sakamoto Yoshinobu Matsuda Chikatoshi Sugimoto Hyung-Eun Yoon Yoshikazu Inoue
author_facet	Teiko Sakurai Toru Arai Masaki Hirose Kensuke Kojima Tetsuki Sakamoto Yoshinobu Matsuda Chikatoshi Sugimoto Hyung-Eun Yoon Yoshikazu Inoue
author_sort	Teiko Sakurai
title	Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
title_short	Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
title_full	Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
title_fullStr	Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
title_full_unstemmed	Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
title_sort	reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis
publisher	BMC
publishDate	2021
url	https://doaj.org/article/fee158e7a1ac487b97531e02ca80e47b
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Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis

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