Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea
Abstract We aimed to describe the clinical features of lymphangioleiomyomatosis (LAM) in Korean patients and identify factors associated with progressive disease (PD). Clinical features of 54 patients with definite or probable LAM from 2005 to 2018 were retrospectively analysed. Common features were...
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| Format: | Article |
| Language: | English |
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Nature Portfolio
2022-05-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-022-12314-1 |
| _version_ | 1818240958449319936 |
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| author | Shihwan Chang Ji Soo Choi Ah Young Leem Su Hwan Lee Sang Hoon Lee Song Yee Kim Kyung Soo Chung Ji Ye Jung Young Ae Kang Young Sam Kim Jin Gu Lee Hyo Chae Paik Hyo Sup Shim Eun Hye Lee Moo Suk Park |
| author_facet | Shihwan Chang Ji Soo Choi Ah Young Leem Su Hwan Lee Sang Hoon Lee Song Yee Kim Kyung Soo Chung Ji Ye Jung Young Ae Kang Young Sam Kim Jin Gu Lee Hyo Chae Paik Hyo Sup Shim Eun Hye Lee Moo Suk Park |
| author_sort | Shihwan Chang |
| collection | DOAJ |
| description | Abstract We aimed to describe the clinical features of lymphangioleiomyomatosis (LAM) in Korean patients and identify factors associated with progressive disease (PD). Clinical features of 54 patients with definite or probable LAM from 2005 to 2018 were retrospectively analysed. Common features were pneumothorax (66.7%) and abdominal lymphadenopathy (50.0%). Twenty-three (42.6%) patients were initially treated with mechanistic target of rapamycin (mTOR) inhibitors. Lung transplantation (LT) was performed in 13 (24.1%) patients. Grouped based on the annual decline in forced expiratory volume in 1 s (FEV1) from baseline and LT, 36 (66.7%) patients exhibited stable disease (SD). All six deaths (11.1%) occurred in PD. Proportion of SD was higher in those treated initially with mTOR inhibitors than in those under observation (p = 0.043). Univariate analysis revealed sirolimus use, and baseline forced vital capacity, FEV1, and diffusing capacity of the lungs for carbon monoxide are associated with PD. Multivariate analysis showed that only sirolimus use (odds ratio 0.141, 95% confidence interval 0.021–0.949, p = 0.044) reduced PD. Kaplan–Meier analysis estimates overall survival of 92.0% and 74.7% at 5 and 10 years, respectively. A considerable proportion of LAM patients remain clinically stable without treatment. LT is an increasingly viable option for patients with severe lung function decline. |
| first_indexed | 2024-12-12T13:21:43Z |
| format | Article |
| id | doaj.art-32165f2c5a0b44ee945541fbd675f0cb |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-12-12T13:21:43Z |
| publishDate | 2022-05-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-32165f2c5a0b44ee945541fbd675f0cb2022-12-22T00:23:17ZengNature PortfolioScientific Reports2045-23222022-05-0112111010.1038/s41598-022-12314-1Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in KoreaShihwan Chang0Ji Soo Choi1Ah Young Leem2Su Hwan Lee3Sang Hoon Lee4Song Yee Kim5Kyung Soo Chung6Ji Ye Jung7Young Ae Kang8Young Sam Kim9Jin Gu Lee10Hyo Chae Paik11Hyo Sup Shim12Eun Hye Lee13Moo Suk Park14Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineDepartment of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University College of MedicineDepartment of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University College of MedicineDepartment of Pathology, Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of MedicineDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of MedicineAbstract We aimed to describe the clinical features of lymphangioleiomyomatosis (LAM) in Korean patients and identify factors associated with progressive disease (PD). Clinical features of 54 patients with definite or probable LAM from 2005 to 2018 were retrospectively analysed. Common features were pneumothorax (66.7%) and abdominal lymphadenopathy (50.0%). Twenty-three (42.6%) patients were initially treated with mechanistic target of rapamycin (mTOR) inhibitors. Lung transplantation (LT) was performed in 13 (24.1%) patients. Grouped based on the annual decline in forced expiratory volume in 1 s (FEV1) from baseline and LT, 36 (66.7%) patients exhibited stable disease (SD). All six deaths (11.1%) occurred in PD. Proportion of SD was higher in those treated initially with mTOR inhibitors than in those under observation (p = 0.043). Univariate analysis revealed sirolimus use, and baseline forced vital capacity, FEV1, and diffusing capacity of the lungs for carbon monoxide are associated with PD. Multivariate analysis showed that only sirolimus use (odds ratio 0.141, 95% confidence interval 0.021–0.949, p = 0.044) reduced PD. Kaplan–Meier analysis estimates overall survival of 92.0% and 74.7% at 5 and 10 years, respectively. A considerable proportion of LAM patients remain clinically stable without treatment. LT is an increasingly viable option for patients with severe lung function decline.https://doi.org/10.1038/s41598-022-12314-1 |
| spellingShingle | Shihwan Chang Ji Soo Choi Ah Young Leem Su Hwan Lee Sang Hoon Lee Song Yee Kim Kyung Soo Chung Ji Ye Jung Young Ae Kang Young Sam Kim Jin Gu Lee Hyo Chae Paik Hyo Sup Shim Eun Hye Lee Moo Suk Park Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea Scientific Reports |
| title | Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea |
| title_full | Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea |
| title_fullStr | Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea |
| title_full_unstemmed | Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea |
| title_short | Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea |
| title_sort | long term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in korea |
| url | https://doi.org/10.1038/s41598-022-12314-1 |
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