诊断学理论与实践

诊断学理论与实践 >

2022 , Vol. 21 >Issue 06: 746 - 750

DOI: https://doi.org/10.16150/j.1671-2870.2022.06.14

综述

肺淋巴管平滑肌瘤病肺部病变的影像学研究进展

展开
  • 上海交通大学医学院附属瑞金医院放射科，上海 200025

收稿日期: 2022-03-28

  网络出版日期: 2023-04-23

基金资助

国家自然科学基金项目(8217070113);国家自然科学基金项目(81501467);上海市中西医临床协作试点建设项目([ZY(2018-2020)-FWTX-1003）])

收起

Advances in imaging study of pulmonary lymphangioleiomyomatosis

Expand
  • Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China

Received date: 2022-03-28

  Online published: 2023-04-23

Fold

摘要

淋巴管平滑肌瘤病（lymphangioleiomyomatosis，LAM）是一种罕见的特发性疾病，其主要累及器官为肺，即肺LAM（pulmonary LAM，PLAM），表现为两肺弥漫肺气囊。近年来PLAM的影像学研究及其进展迅速，包括CT图像中的肺气囊定量、纹理分析，PET-CT显示肺实质及淋巴病变等。基于CT的PLAM肺气囊半定量、全定量、组学分析等，不仅能够精准地反映PLAM患者肺内病变的范围，且与肺功能关联度高，为患者病情评估、疗效预测等提供了新方法。PET/CT及SPECT/CT能够较好地评估PLAM患者的淋巴系统病变情况。

关键词： 淋巴管平滑肌瘤病; 影像学; 定量

本文引用格式

立克拉虎, 杨文洁 . 肺淋巴管平滑肌瘤病肺部病变的影像学研究进展[J]. 诊断学理论与实践, 2022 , 21(06) : 746 -750 . DOI: 10.16150/j.1671-2870.2022.06.14

Abstract

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare idiopathic disease, which mainly involves lung and presents with diffused lung cysts. This paper reviews the imaging study and research progress of PLAM in recent years, including the quantitative analysis, texture analysis in CT images based pulmonary balloon. PET/CT may shows lesions in pulmonary parenchyma or lymphatic system. The semi-quantitative, full quantitative and omics analysis of pulmonary balloons based on CT could reflect the range of pulmonary lesions, and has a positive correlation with pulmonary function, which provide new methods for disease assessment and curative efficacy prediction in the patients with PLAM. The PET/CT and SPECT/CT have better evaluation effectiveness for the lymphoid system lesions in PLAM patients

Key words： Lymphangioleiomyomatosis; Imaging; Quantification

参考文献

[1] Harknett E C, Chang W Y, Byrnes S, et al. Use of variabi-lity in national and regional data to estimate the prevalence of lymphangioleiomyomatosis[J]. QJM, 2011, 104(11):971-979.
[2] Mavroudi M, Zarogoulidis P, Katsikogiannis N, et al. Lymphangioleiomyomatosis: current and future[J]. J Thorac Dis, 2013, 5(1):74-79.
[3] Avila N A, Dwyer A J, Rabel A, et al. Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features[J]. Radiology, 2007, 242(1):277-285.
[4] Johnson S R, Taveira-DaSilva A M, Moss J. Lymphangio-leiomyomatosis[J]. Clin Chest Med, 2016, 37(3):389-403.
[5] Taveiradasilva A M, Moss J. Epidemiology, pathogenesis and diagnosis of lymphangioleiomyomatosis[J]. Expert Opin Orphan Drugs, 2016, 4(4):369-378.
[6] Watanabe R, Wei L, Huang J. mTOR signaling, function, novel inhibitors, and therapeutic targets[J]. J Nucl Med, 2011, 52(4):497-500.
[7] McCormack F X, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis[J]. N Engl J Med, 2011, 364(17):1595-1606.
[8] 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.
[9] McCormack F X, Gupta N, Finlay G R, 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] Gupta N, Finlay G A, Kotloff R M, et al. Lymphangio-leiomyomatosis 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.
[11] Steagall W K, Glasgow C G, Hathaway O M, et al. Genetic and morphologic determinants of pneumothorax in lymphangioleiomyomatosis[J]. Am J Physiol Lung Cell Mol Physiol, 2007, 293(3):L800-L808.
[12] Theilig D, Doellinger F, Kuhnigk J M, et al. Pulmonary lymphangioleiomyomatosis: analysis of disease manifestation by region-based quantification of lung parenchyma[J]. Eur J Radiol, 2015, 84(4):732-737.
[13] Müller N L, Chiles C, Kullnig P. Pulmonary lymphangiomyomatosis: correlation of CT with radiographic and functional findings[J]. Radiology, 1990, 175(2):335-339.
[14] Gupta N, Lee H S, Ryu JH, et al. The NHLBI LAM Re-gistry: Prognostic Physiologic and Radiologic Biomarkers Emerge From a 15-Year Prospective Longitudinal Analysis[J]. Chest, 2019, 155(2):288-296.
[15] 吴喜端, 曾庆思, 黄绥丹, 等. HRCT扫描对肺淋巴管平滑肌瘤病的肺部结构研究[J]. 广州医科大学学报, 2017, 45(5):5-8.
[15] Wu X R, Zeng Q S, Huang S D, et al. Lung structural assessment in pulmonary lymphangioleiomyomatosis using HRCT[J]. Acad J Guangzhou Med Coll, 2017, 45(5):5-8.
[16] van Rikxoort E M, de Hoop B, van de Vorst S, et al. Automatic segmentation of pulmonary segments from volumetric chest CT scans[J]. IEEE Trans Med Imaging, 2009, 28(4):621-630.
[17] Gopalakrishnan V, Yao J, Steagall W K, et al. Use of CT Imaging to Quantify Progression and Response to Treatment in Lymphangioleiomyomatosis[J]. Chest, 2019, 155(5):962-971.
[18] 宋惜夕, 王坚, 蔡慧, 等. 利用定量CT评估淋巴管平滑肌瘤病的肺部损害[J]. 复旦学报(医学版), 2021, 48(5):653-659.
[18] Song X X, Wang J, Cai H, et al. Usage of quantitative CT in evaluating the degree of lung destruction in lymphangioleiomyomatosis[J]. Fudan Univ J of Med Sci, 2021, 48(5):653-659.
[19] Matthew B P, Hasani A M, Chen YC, et al. Ultra-Small Lung Cysts Impair Diffusion Without Obstructing Air Flow in Lymphangioleiomyomatosis[J]. Chest, 2021, 160(1):199-208.
[20] Ni Z, Ng TSC, Liu J, et al. Quantitative assessment of pulmonary function in lymphangioleiomyomatosis patients using high-resolution computed tomography and pulmonary function tests[J]. J Thorac Dis, 2020, 12(11):6466-6475.
[21] Yao J, Taveira-DaSilva A M, Colby T V, et al. CT grading of lung disease in lymphangioleiomyomatosis[J]. AJR Am J Roentgenol, 2012, 199(4):787-793.
[22] Hu-Wang E, Schuzer J L, Rollison S, et al. Chest CT Scan at Radiation Dose of a Posteroanterior and Lateral Chest Radiograph Series: A Proof of Principle in Lymphangio-leiomyomatosis[J]. Chest, 2019, 155(3):528-533.
[23] Gopalakrishnan V, Yao J, Steagall W K, et al. Use of CT Imaging to Quantify Progression and Response to Treatment in Lymphangioleiomyomatosis[J]. Chest, 2019, 155(5):962-971.
[24] Yao J, Taveira-DaSilva AM, Colby TV, et al. CT grading of lung disease in lymphangioleiomyomatosis[J]. AJR Am J Roentgenol, 2012, 199(4):787-793.
[25] Yao J, Taveira-DaSilva AM, Jones AM, et al. Sustained effects of sirolimus on lung function and cystic lung lesions in lymphangioleiomyomatosis[J]. Am J Respir Crit Care Med, 2014, 190(11):1273-1282.
[26] Ando K, Tobino K, Kurihara M, et al. Quantitative CT analysis of small pulmonary vessels in lymphangioleiomyomatosis[J]. Eur J Radiol, 2012, 81(12):3925-3930.
[27] Jonas A, Muelly M, Gupta N, et al. Machine learning to distinguish lymphangioleiomyomatosis from other diffuse cystic lung diseases[J]. Respir Investig, 2022, 60(3):430-433.
[28] Hou G, Xu W, Jiang Y, et al. Lymphangioleiomyomatosis revealed by 68Ga-NOTA-Evans Blue PET/CT[J]. Eur J Nucl Med Mol Imaging, 2020, 47(10):2469-2470.
[29] Hou G, Jiang Y, Jing H, et al. Usefulness of 99mTc-ASC lymphoscintigraphy and SPECT/CT in the evaluation of rare lymphatic disorders: Gorham-Stout disease, lymphangioma, and lymphangioleiomyomatosis[J]. Medicine (Baltimore), 2020, 99(39):e22414.
[30] Hou G, Jiang Y, Xu W, et al. 68Ga-NOTA-Evans Blue PET/CT findings in lymphangioleiomyomatosis compared with 99mTC-ASC lymphoscintigraphy: a prospective study[J]. Orphanet J Rare Dis, 2021, 16(1):279.
Options
文章导航

/

〈 〉