PMID- 35585116 OWN - NLM STAT- MEDLINE DCOM- 20220520 LR - 20220716 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 12 IP - 1 DP - 2022 May 18 TI - Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea. PG - 8260 LID - 10.1038/s41598-022-12314-1 [doi] LID - 8260 AB - 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 (FEV(1)) 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, FEV(1), 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. CI - (c) 2022. The Author(s). FAU - Chang, Shihwan AU - Chang S AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Choi, Ji Soo AU - Choi JS AD - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, 363 Dongbaekjukjeon-daero, Giheung-gu, Yongin, 16995, Republic of Korea. FAU - Leem, Ah Young AU - Leem AY AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Lee, Su Hwan AU - Lee SH AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Lee, Sang Hoon AU - Lee SH AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Kim, Song Yee AU - Kim SY AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Chung, Kyung Soo AU - Chung KS AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Jung, Ji Ye AU - Jung JY AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Kang, Young Ae AU - Kang YA AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Kim, Young Sam AU - Kim YS AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Lee, Jin Gu AU - Lee JG AD - Department of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Paik, Hyo Chae AU - Paik HC AD - Department of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Shim, Hyo Sup AU - Shim HS AD - Department of Pathology, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. FAU - Lee, Eun Hye AU - Lee EH AD - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, 363 Dongbaekjukjeon-daero, Giheung-gu, Yongin, 16995, Republic of Korea. hieunhye@yuhs.ac. FAU - Park, Moo Suk AU - Park MS AD - Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea. PMS70@yuhs.ac. LA - eng PT - Journal Article DEP - 20220518 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - W36ZG6FT64 (Sirolimus) SB - IM MH - Forced Expiratory Volume MH - Humans MH - *Lung Neoplasms/drug therapy MH - *Lung Transplantation MH - *Lymphangioleiomyomatosis/drug therapy/surgery MH - Referral and Consultation MH - Retrospective Studies MH - Sirolimus/therapeutic use PMC - PMC9117329 COIS- The authors declare no competing interests. EDAT- 2022/05/19 06:00 MHDA- 2022/05/21 06:00 PMCR- 2022/05/18 CRDT- 2022/05/18 23:19 PHST- 2021/10/01 00:00 [received] PHST- 2022/04/26 00:00 [accepted] PHST- 2022/05/18 23:19 [entrez] PHST- 2022/05/19 06:00 [pubmed] PHST- 2022/05/21 06:00 [medline] PHST- 2022/05/18 00:00 [pmc-release] AID - 10.1038/s41598-022-12314-1 [pii] AID - 12314 [pii] AID - 10.1038/s41598-022-12314-1 [doi] PST - epublish SO - Sci Rep. 2022 May 18;12(1):8260. doi: 10.1038/s41598-022-12314-1.