PMID- 34732225 OWN - NLM STAT- MEDLINE DCOM- 20211111 LR - 20211111 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 16 IP - 1 DP - 2021 Nov 3 TI - Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis. PG - 466 LID - 10.1186/s13023-021-02081-z [doi] LID - 466 AB - 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. CI - (c) 2021. The Author(s). FAU - Sakurai, Teiko AU - Sakurai T AD - Department of General Thoracic Surgery, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Arai, Toru AU - Arai T AUID- ORCID: 0000-0002-2222-0683 AD - Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Hirose, Masaki AU - Hirose M AD - Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Kojima, Kensuke AU - Kojima K AD - Department of General Thoracic Surgery, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Sakamoto, Tetsuki AU - Sakamoto T AD - Department of General Thoracic Surgery, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Matsuda, Yoshinobu AU - Matsuda Y AUID- ORCID: 0000-0001-5092-9377 AD - Department of Psychosomatic Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Sugimoto, Chikatoshi AU - Sugimoto C AD - Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Yoon, Hyung-Eun AU - Yoon HE AD - Department of General Thoracic Surgery, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. FAU - Inoue, Yoshikazu AU - Inoue Y AUID- ORCID: 0000-0003-3994-874X AD - Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Nagasone-cho 1180, Sakai, Osaka, 591-8555, Japan. giichiyi@me.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211103 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - W36ZG6FT64 (Sirolimus) SB - IM MH - Humans MH - Lung MH - *Lung Neoplasms MH - *Lymphangioleiomyomatosis/drug therapy/surgery MH - Neoplasm Recurrence, Local/drug therapy MH - *Pneumothorax/drug therapy/etiology/surgery MH - Retrospective Studies MH - Sirolimus/therapeutic use PMC - PMC8567719 OTO - NOTNLM OT - Lymphangioleiomyomatosis OT - Pneumothorax OT - Sirolimus OT - Surgical pleural covering OT - Total pleural covering OT - Vascular endothelial growth factor D COIS- TA reports personal fees from Boehringer Ingelheim and from Shionogi Co Ltd outside the submitted work; YI reports grants from the Ministry of Health, Labor and Welfare, Japan, personal fees, and other fees from Boehringer Ingelheim, Taiho, Roche, Shionogi, Galapagos, and Savara outside the submitted work. EDAT- 2021/11/05 06:00 MHDA- 2021/11/12 06:00 PMCR- 2021/11/03 CRDT- 2021/11/04 05:29 PHST- 2021/06/05 00:00 [received] PHST- 2021/10/10 00:00 [accepted] PHST- 2021/11/04 05:29 [entrez] PHST- 2021/11/05 06:00 [pubmed] PHST- 2021/11/12 06:00 [medline] PHST- 2021/11/03 00:00 [pmc-release] AID - 10.1186/s13023-021-02081-z [pii] AID - 2081 [pii] AID - 10.1186/s13023-021-02081-z [doi] PST - epublish SO - Orphanet J Rare Dis. 2021 Nov 3;16(1):466. doi: 10.1186/s13023-021-02081-z.