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Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea

 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 
 SCIENTIFIC REPORTS, Vol.12(1) : 8260, 2022-05 
Journal Title
Issue Date
Forced Expiratory Volume ; Humans ; Lung Neoplasms* / drug therapy ; Lung Transplantation* ; Lymphangioleiomyomatosis* / drug therapy ; Lymphangioleiomyomatosis* / surgery ; Referral and Consultation ; Retrospective Studies ; Sirolimus / therapeutic use
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.
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Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Thoracic and Cardiovascular Surgery (흉부외과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Young Ae(강영애) ORCID logo https://orcid.org/0000-0002-7783-5271
Kim, Song Yee(김송이) ORCID logo https://orcid.org/0000-0001-8627-486X
Kim, Young Sam(김영삼) ORCID logo https://orcid.org/0000-0001-9656-8482
Park, Moo Suk(박무석) ORCID logo https://orcid.org/0000-0003-0820-7615
Paik, Hyo Chae(백효채) ORCID logo https://orcid.org/0000-0001-9309-8235
Shim, Hyo Sup(심효섭) ORCID logo https://orcid.org/0000-0002-5718-3624
Lee, Sang Hoon(이상훈) ORCID logo https://orcid.org/0000-0002-7706-5318
Lee, Su Hwan(이수환) ORCID logo https://orcid.org/0000-0002-3487-2574
Lee, Eun Hye(이은혜) ORCID logo https://orcid.org/0000-0003-2570-3442
Lee, Jin Gu(이진구)
Leem, Ah Young(임아영) ORCID logo https://orcid.org/0000-0001-5165-3704
Jung, Kyung Soo(정경수) ORCID logo https://orcid.org/0000-0003-1604-8730
Jung, Ji Ye(정지예) ORCID logo https://orcid.org/0000-0003-1589-4142
Choi, Ji Soo(최지수) ORCID logo https://orcid.org/0000-0003-2716-6775
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