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中华临床医师杂志(电子版) ›› 2021, Vol. 15 ›› Issue (07) : 556 -560. doi: 10.3877/cma.j.issn.1674-0785.2021.07.015

综述

肺淋巴管肌瘤病治疗的研究进展
陈雪芬1, 邓静敏1,()   
  1. 1. 530021 南宁,广西医科大学第一附属医院呼吸与危重症医学科
  • 收稿日期:2021-03-02 出版日期:2021-07-15
  • 通信作者: 邓静敏

Progress in treatment of pulmonary lymphangiomyomatosis

Xuefen Chen1, Jingmin Deng1,()   

  1. 1. Department of Respiratory Medicine, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China
  • Received:2021-03-02 Published:2021-07-15
  • Corresponding author: Jingmin Deng
引用本文:

陈雪芬, 邓静敏. 肺淋巴管肌瘤病治疗的研究进展[J]. 中华临床医师杂志(电子版), 2021, 15(07): 556-560.

Xuefen Chen, Jingmin Deng. Progress in treatment of pulmonary lymphangiomyomatosis[J]. Chinese Journal of Clinicians(Electronic Edition), 2021, 15(07): 556-560.

肺淋巴管肌瘤病(PLAM)是一种罕见的肺部疾病,好发于育龄期女性,主要临床表现为反复气胸、乳糜胸、呼吸困难等,典型影像学表现为两肺弥漫性囊性病变,治疗较困难,患者预后差。PLAM的治疗包括药物治疗、气胸及乳糜胸的处理、肺移植等,其中西罗莫司是主要的治疗药物。本文就PLAM的治疗研究进展进行综述,以期为临床治疗及后续研究提供参考。

Pulmonary lymphangiomyomatosis is a rare lung disease that occurs in women of reproductive age. Main clinical manifestations are repeated pneumothorax, chylothorax, dyspnea and so on. Typical imaging manifestation is diffuse cystic lesions of both lungs. Clinical treatment is difficult, and prognosis is poor. Treatments for PLAM includes drugs treatment, managment of pneumothorax and chylothorax, and lung transplantation. Among them, Sirolimus is the main therapeutic drug. This article reviews the progress in treatment of PLAM to provide references for clinical treatment and future research of this disease.

1
Kitaichi M, Nishimura K, Itoh H, et al. Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors [J]. Am J Respir Crit Care Med, 1995, 151(2 Pt 1): 527-533.
2
Taylor JR, Ryu J, Colby TV, et al. Lymphangioleiomyomatosis. Clinical course in 32 patients [J]. N Engl J Med,1990, 323(18): 1254-1260.
3
Gupta N, Lee HS, Ryu JH, et al. The NHLBI LAM registry: prognostic physiologic and radiologic biomarkers emerge from a 15-year prospective longitudinal analysis [J]. Chest, 2019, 155(2): 288-296.
4
Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis diagnosis and management: high-resolution chest computed tomography, transbronchial lung biopsy, and pleural disease management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline [J]. Am J Respir Crit Care Med, 2017, 196(10): 1337-1348.
5
Krymskaya VP, McCormack FX. Lymphangioleiomyomatosis: a monogenic model of malignancy [J]. Annu Rev Med, 2017, 68: 69-83.
6
Goncharova EA, Goncharov DA, Li H, et al. mTORC2 is required for proliferation and survival of TSC2-null cells [J]. Mol Cell Biol, 2011, 31(12): 2484-2498.
7
Kim W, Giannikou K, Dreier JR, et al. A genome-wide association study implicates NR2F2 in lymphangioleiomyomatosis pathogenesis [J]. Eur Respir J, 2019, 53(6): 1900329.
8
Kida Y. Efficacy and safety of sirolimus in lymphangioleiomyomatosis [J]. N Engl J Med, 2011, 365(3): 271.
9
McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: lymphangioleiomyomatosis diagnosis and management [J]. Am J Respir Crit Care Med, 2016, 194(6): 748-761.
10
Li X, Liu X, Zhang L, et al. Insulin growth factor binding protein 2 mediates the progression of lymphangioleiomyomatosis [J]. Oncotarget, 2017, 8(22): 36628-36638.
11
Himes BE, Obraztsova K, Lian L, et al. Rapamycin-independent IGF2 expression in Tsc2-null mouse embryo fibroblasts and human lymphangioleiomyomatosis cells [J]. PLoS One, 2018, 13(5): e0197105.
12
Parkhitko A, Myachina F, Morrison TA, et al. Tumorigenesis in tuberous sclerosis complex is autophagy and p62/sequestosome 1 (SQSTM1)-dependent [J]. Proc Natl Acad Sci U S A, 2011, 108(30): 12455-12460.
13
Zhan Y, Shen L, Xu W, et al. Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study [J]. Orphanet J Rare Dis, 2018, 13(1): 34.
14
Ando K, Kurihara M, Kataoka H, et al. Efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis [J]. Respir Investig, 2013, 51(3): 175-183.
15
Yoon HY, Hwang JJ, Kim DS, et al. Efficacy and safety of low-dose Sirolimus in Lymphangioleiomyomatosis [J]. Orphanet J Rare Dis, 2018, 13(1): 204.
16
淋巴管肌瘤病共识专家组. 西罗莫司治疗淋巴管肌瘤病专家共识(2018) [J]. 中华结核和呼吸杂志, 2019, 42(2): 92-97.
17
Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment [J]. Am J Respir Crit Care Med, 2006, 173(1): 105-111.
18
Guardia O, Rial Mdel C, Casadei D. Pregnancy under sirolimus-based immunosuppression [J]. Transplantation, 2006, 81(4): 636.
19
Landh E, Moir LM, Gomes Dos Reis L, et al. Inhaled rapamycin solid lipid nano particles for the treatment of lymphangioleiomyomatosis [J]. Eur J Pharm Sci, 2020, 142: 105098.
20
Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis [J]. Eur Respir J, 2010, 35(1): 14-26.
21
Lu C, Lee HS, Pappas GP, et al. A phase II clinical trial of an aromatase inhibitor for postmenopausal women with lymphangioleiomyomatosis [J]. Ann Am Thorac Soc, 2017, 14(6): 919-928.
22
El-Chemaly S, Taveira-Dasilva A, Goldberg HJ, et al. Sirolimus and autophagy inhibition in lymphangioleiomyomatosis: results of a phase I clinical trial [J]. Chest, 2017, 151(6): 1302-1310.
23
El-Chemaly S, Taveira-DaSilva A, Stylianou MP, et al. Statins in lymphangioleiomyomatosis: a word of caution [J]. Eur Respir J, 2009, 34(2): 513-514.
24
Krymskaya VP, Courtwright AM, Fleck V, et al. A phase II clinical trial of the safety of simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex [J]. Respir Med, 2020, 163: 105898.
25
Pimenta SP, Baldi BG, Acencio MM, et al. Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade [J]. J Bras Pneumol, 2011, 37(4): 424-430.
26
Moses MA, Harper J, and Folkman J. Doxycycline treatment for lymphangioleiomyomatosis with urinary monitoring for MMPs [J]. N Engl J Med, 2006, 354(24): 2621-2622.
27
Kim SH, Baek MS, Yoon DS, et al. Vitamin D inhibits expression and activity of matrix metalloproteinase in human lung fibroblasts (HFL-1) cells [J]. Tuberc Respir Dis (Seoul), 2014, 77(2): 73-80.
28
Chang WY, Cane JL, Kumaran M, et al. A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis [J]. Eur Respir J, 2014, 43(4): 1114-1123.
29
Le K, Steagall WK, Stylianou M, et al. Effect of beta-agonists on LAM progression and treatment [J]. Proc Natl Acad Sci U S A, 2018, 115(5): E944-E953.
30
Johnson J, Johnson SR. Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use [J]. Thorax, 2019, 74(10): 999-1002.
31
Hu S, Wu X, Xu W, et al. Long-term efficacy and safety of sirolimus therapy in patients with lymphangioleiomyomatosis [J]. Orphanet J Rare Dis, 2019, 14(1): 206.
32
Gupta N, Lee HS, Young LR, et al. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis [J]. Eur Respir J, 2019, 53(4): 1802066.
33
Almoosa KF, Ryu JH, Mendez J, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications [J]. Chest, 2006, 129(5): 1274-1281.
34
Kurihara M, Mizobuchi T, Kataoka H, et al. A total pleural covering for lymphangioleiomyomatosis prevents pneumothorax recurrence [J]. PLoS One, 2016, 11(9): e0163637.
35
Taveira-DaSilva AM, Jones AM, Julien-Williams P, et al. Severity and outcome of cystic lung disease in women with tuberous sclerosis complex [J]. Eur Respir J, 2015, 45(1): 171-180.
36
Boehm PM, Ghanim BD, Matilla J, et al. Chylothorax in a 32-year-old patient with lymphangioleiomyomatosis after bilateral lung transplant [J]. Ann Thorac Surg, 2018, 105(6): e263-e264.
37
Bee J, Fuller S, Miller S, et al. Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study [J]. Thorax, 2018, 73(4): 369-375.
38
Fine NM, Kushwaha SS. Recent advances in mammalian target of rapamycin inhibitor use in heart and lung transplantation [J]. Transplantation, 2016, 100(12): 2558-2568.
39
El-Chemaly S, Goldberg HJ, Glanville AR. Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation? [J]. Expert Rev Respir Med, 2014, 8(6): 657-660.
40
Khawar MU, Yazdani D, Zhu Z, et al. Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis [J]. J Heart Lung Transplant, 2019, 38(9): 949-955.
41
Salman J, Ius F, Sommer W, et al. Long-term results of bilateral lung transplantation in patients with end-stage pulmonary lymphangioleiomyomatosis [J]. Prog Transplant, 2019, 29(2): 115-121.
42
Li X, Xu W, Zhang L, et al. Effects of yoga on exercise capacity in patients with lymphangioleiomyomatosis: a nonrandomized controlled study [J]. Orphanet J Rare Dis, 2020, 15(1): 72.
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