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 regressi...
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| Format: | Article |
| Language: | English |
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BMC
2022-07-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-022-02418-2 |
| _version_ | 1818519297969881088 |
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| author | Chongsheng Cheng Wenshuai Xu Yani Wang Tengyue Zhang Luning Yang Wangji Zhou Danjing Hu Yanli Yang Xinlun Tian Kai-Feng Xu |
| author_facet | Chongsheng Cheng Wenshuai Xu Yani Wang Tengyue Zhang Luning Yang Wangji Zhou Danjing Hu Yanli Yang Xinlun Tian Kai-Feng Xu |
| author_sort | Chongsheng Cheng |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-11T01:22:18Z |
| format | Article |
| id | doaj.art-a1d8f4aa47134731b76c59c1b25b631f |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-11T01:22:18Z |
| publishDate | 2022-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-a1d8f4aa47134731b76c59c1b25b631f2022-12-22T01:25:40ZengBMCOrphanet Journal of Rare Diseases1750-11722022-07-011711710.1186/s13023-022-02418-2Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled studyChongsheng Cheng0Wenshuai Xu1Yani Wang2Tengyue Zhang3Luning Yang4Wangji Zhou5Danjing Hu6Yanli Yang7Xinlun Tian8Kai-Feng Xu9State Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesState Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical SciencesAbstract 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.https://doi.org/10.1186/s13023-022-02418-2LymphangioleiomyomatosisPneumothoraxRecurrenceSirolimus |
| spellingShingle | Chongsheng Cheng Wenshuai Xu Yani Wang Tengyue Zhang Luning Yang Wangji Zhou Danjing Hu Yanli Yang Xinlun Tian Kai-Feng Xu Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study Orphanet Journal of Rare Diseases Lymphangioleiomyomatosis Pneumothorax Recurrence Sirolimus |
| title | Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study |
| title_full | Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study |
| title_fullStr | Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study |
| title_full_unstemmed | Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study |
| title_short | Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study |
| title_sort | sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis a historical prospective self controlled study |
| topic | Lymphangioleiomyomatosis Pneumothorax Recurrence Sirolimus |
| url | https://doi.org/10.1186/s13023-022-02418-2 |
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