Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Sporadic lymphangioleiomyomatosis: Treatment and prognosis

Francis X McCormack, MD
Nishant Gupta, MD
Section Editor
Talmadge E King, Jr, MD
Deputy Editors
Geraldine Finlay, MD
Helen Hollingsworth, MD


Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder that mostly afflicts women. The term sporadic LAM is used for patients with LAM who do not have tuberous sclerosis complex (TSC), while TSC-LAM refers to LAM that occurs in patients with TSC. The management of LAM is generally based upon supportive care, use of sirolimus to slow progression of respiratory impairment, and prevention or treatment of complications [1,2].

The management, monitoring, and prognosis of sporadic LAM and its complications are reviewed here. The epidemiology, pathogenesis, clinical presentation, and diagnostic evaluation are discussed separately. (See "Sporadic lymphangioleiomyomatosis: Epidemiology and pathogenesis" and "Sporadic lymphangioleiomyomatosis: Clinical presentation and diagnostic evaluation".)


Given the complexities and multisystem nature of LAM, we encourage patient referral to centers with expertise in this disorder for shared care between LAM experts and local pulmonologists. (See 'Additional resources' below.)

Supportive care — Several supportive measures are useful in managing patients with LAM:

Avoidance of cigarette smoking – Patients with LAM should be advised to avoid cigarette smoking and passive exposure to tobacco smoke, given the potential for accelerating disease progression (table 1). (See "Overview of smoking cessation management in adults" and "Pharmacotherapy for smoking cessation in adults" and "Behavioral approaches to smoking cessation".)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Nov 22, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J 2010; 35:14.
  2. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. Am J Respir Crit Care Med 2016; 194:748.
  3. Medeiros P Jr, Lorenzi-Filho G, Pimenta SP, et al. Sleep desaturation and its relationship to lung function, exercise and quality of life in LAM. Respir Med 2012; 106:420.
  4. Bahmer T, Watz H, Waschki B, et al. Reduced physical activity in lymphangioleiomyomatosis compared with COPD and healthy controls: disease-specific impact and clinical correlates. Thorax 2016; 71:662.
  5. Araujo MS, Baldi BG, Freitas CS, et al. Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial. Eur Respir J 2016; 47:1452.
  6. Fritz H, Seely D, Flower G, et al. Soy, red clover, and isoflavones and breast cancer: a systematic review. PLoS One 2013; 8:e81968.
  7. Taveira-DaSilva AM, Hedin C, Stylianou MP, et al. Reversible airflow obstruction, proliferation of abnormal smooth muscle cells, and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis. Am J Respir Crit Care Med 2001; 164:1072.
  8. Burger CD. Variability in the prevalence of acute bronchoresponsiveness in different populations of patients with lymphangioleiomyomatosis. Chest 2008; 134:217; author reply 218.
  9. Baldi BG, de Albuquerque AL, Pimenta SP, et al. A pilot study assessing the effect of bronchodilator on dynamic hyperinflation in LAM. Respir Med 2013; 107:1773.
  10. Taveira-DaSilva AM, Burstein D, Hathaway OM, et al. Pneumothorax after air travel in lymphangioleiomyomatosis, idiopathic pulmonary fibrosis, and sarcoidosis. Chest 2009; 136:665.
  11. Pollock-BarZiv S, Cohen MM, Downey GP, et al. Air travel in women with lymphangioleiomyomatosis. Thorax 2007; 62:176.
  12. American College of Obstetricians and Gynecologists’ Committee on Gynecologic Practice, Farrell R. ACOG Committee Opinion No. 659: The Use of Vaginal Estrogen in Women With a History of Estrogen-Dependent Breast Cancer. Obstet Gynecol 2016; 127:e93.
  13. Johnson SR, Tattersfield AE. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000; 55:1052.
  14. Cleary-Goldman J, Sanghvi AV, Nakhuda GS, Robinson JN. Conservative management of pulmonary lymphangioleiomyomatosis and tuberous sclerosis complicated by renal angiomyolipomas in pregnancy. J Matern Fetal Neonatal Med 2004; 15:132.
  15. Brunelli A, Catalini G, Fianchini A. Pregnancy exacerbating unsuspected mediastinal lymphangioleiomyomatosis and chylothorax. Int J Gynaecol Obstet 1996; 52:289.
  16. Mitra S, Ghosal AG, Bhattacharya P. Pregnancy unmasking lymphangioleiomyomatosis. J Assoc Physicians India 2004; 52:828.
  17. Fujimoto M, Ohara N, Sasaki H, et al. Pregnancy complicated with pulmonary lymphangioleiomyomatosis: case report. Clin Exp Obstet Gynecol 2005; 32:199.
  18. McLoughlin L, Thomas G, Hasan K. Pregnancy and lymphangioleiomyomatosis: anaesthetic management. Int J Obstet Anesth 2003; 12:40.
  19. Cohen MM, Freyer AM, Johnson SR. Pregnancy experiences among women with lymphangioleiomyomatosis. Respir Med 2009; 103:766.
  20. Raft J, Lalot JM, Meistelman C, Longrois D. [Renal angiomyolipoma rupture during pregnancy]. Gynecol Obstet Fertil 2006; 34:917.
  21. Storm DW, Mowad JJ. Conservative management of a bleeding renal angiomyolipoma in pregnancy. Obstet Gynecol 2006; 107:490.
  22. Morales JP, Georganas M, Khan MS, et al. Embolization of a bleeding renal angiomyolipoma in pregnancy: case report and review. Cardiovasc Intervent Radiol 2005; 28:265.
  23. Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med 2006; 173:105.
  24. Yano S. Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment. Thorax 2002; 57:1085.
  25. Shen A, Iseman MD, Waldron JA, King TE. Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous estrogens. Chest 1987; 91:782.
  26. Wilson AM, Slack HL, Soosay SA, et al. Lymphangioleiomyomatosis. A series of three case reports illustrating the link with high oestrogen states. Scott Med J 2001; 46:150.
  27. Oberstein EM, Fleming LE, Gómez-Marin O, Glassberg MK. Pulmonary lymphangioleiomyomatosis (LAM): examining oral contraceptive pills and the onset of disease. J Womens Health (Larchmt) 2003; 12:81.
  28. Faehling M, Wienhausen-Wilke V, Fallscheer S, et al. Long-term stable lung function and second uncomplicated pregnancy on sirolimus in lymphangioleiomyomatosis (LAM). Sarcoidosis Vasc Diffuse Lung Dis 2015; 32:259.
  29. Faehling M, Frohnmayer S, Leschke M, et al. Successful pregnancy complicated by persistent pneumothorax in a patient with lymphangioleiomyomatosis (LAM) on sirolimus. Sarcoidosis Vasc Diffuse Lung Dis 2011; 28:153.
  30. Jankowska I, Oldakowska-Jedynak U, Jabiry-Zieniewicz Z, et al. Absence of teratogenicity of sirolimus used during early pregnancy in a liver transplant recipient. Transplant Proc 2004; 36:3232.
  31. Cai X, Pacheco-Rodriguez G, Haughey M, et al. Sirolimus decreases circulating lymphangioleiomyomatosis cells in patients with lymphangioleiomyomatosis. Chest 2014; 145:108.
  32. Cai X, Pacheco-Rodriguez G, Fan QY, et al. Phenotypic characterization of disseminated cells with TSC2 loss of heterozygosity in patients with lymphangioleiomyomatosis. Am J Respir Crit Care Med 2010; 182:1410.
  33. Harari S, Elia D, Torre O, et al. Sirolimus Therapy for Patients With Lymphangioleiomyomatosis Leads to Loss of Chylous Ascites and Circulating LAM Cells. Chest 2016; 150:e29.
  34. Eder AF, Ryu JH, Moss J. Should lymphangioleiomyomatosis patients be permitted to donate blood? Eur Respir J 2016; 47:1883.
  35. Young L, Lee HS, Inoue Y, et al. Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. Lancet Respir Med 2013; 1:445.
  36. Radzikowska E, Jaguś P, Sobiecka M, et al. Correlation of serum vascular endothelial growth factor-D concentration with clinical presentation and course of lymphangioleiomyomatosis. Respir Med 2015; 109:1469.
  37. U.S. Food and Drug Administration. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm448523.htm?source=govdelivery&utm_medium=email&utm_source=govdelivery#.VWeLfP4WDY8.facebook (Accessed on May 29, 2015).
  38. McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med 2011; 364:1595.
  39. Ando K, Kurihara M, Kataoka H, et al. Efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis. Respir Investig 2013; 51:175.
  40. Bhorade S, Ahya VN, Baz MA, et al. Comparison of sirolimus with azathioprine in a tacrolimus-based immunosuppressive regimen in lung transplantation. Am J Respir Crit Care Med 2011; 183:379.
  41. Shitrit D, Rahamimov R, Gidon S, et al. Use of sirolimus and low-dose calcineurin inhibitor in lung transplant recipients with renal impairment: results of a controlled pilot study. Kidney Int 2005; 67:1471.
  42. Taveira-DaSilva AM, Hathaway O, Stylianou M, Moss J. Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus. Ann Intern Med 2011; 154:797.
  43. Yao J, Taveira-DaSilva AM, Jones AM, et al. Sustained effects of sirolimus on lung function and cystic lung lesions in lymphangioleiomyomatosis. Am J Respir Crit Care Med 2014; 190:1273.
  44. McCormack FX. Chronic sirolimus therapy for lymphangioleiomyomatosis. Am J Respir Crit Care Med 2014; 190:1332.
  45. Takada T, Mikami A, Kitamura N, et al. Efficacy and Safety of Long-Term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis. Ann Am Thorac Soc 2016; 13:1912.
  46. Bissler JJ, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med 2008; 358:140.
  47. Moua T, Olson EJ, Jean HC, Ryu JH. Resolution of chylous pulmonary congestion and respiratory failure in lymphangioleiomyomatosis with sirolimus therapy. Am J Respir Crit Care Med 2012; 186:389.
  48. Davies DM, Johnson SR, Tattersfield AE, et al. Sirolimus therapy in tuberous sclerosis or sporadic lymphangioleiomyomatosis. N Engl J Med 2008; 358:200.
  49. Dabora SL, Franz DN, Ashwal S, et al. Multicenter phase 2 trial of sirolimus for tuberous sclerosis: kidney angiomyolipomas and other tumors regress and VEGF- D levels decrease. PLoS One 2011; 6:e23379.
  50. Montero MA, Roman A, Berastegui C. Radical reduction of smooth muscle cells in explanted lung of a LAM patient treated with sirolimus: first case report. J Heart Lung Transplant 2012; 31:439.
  51. Neurohr C, Hoffmann AL, Huppmann P, et al. Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? Respir Res 2011; 12:66.
  52. de Oliveira MA, Martins E Martins F, Wang Q, et al. Clinical presentation and management of mTOR inhibitor-associated stomatitis. Oral Oncol 2011; 47:998.
  53. Boers-Doets CB, Raber-Durlacher JE, Treister NS, et al. Mammalian target of rapamycin inhibitor-associated stomatitis. Future Oncol 2013; 9:1883.
  54. Subramaniam S, Zell JA, Kunz PL. Everolimus causing severe hypertriglyceridemia and acute pancreatitis. J Natl Compr Canc Netw 2013; 11:5.
  55. Courtwright AM, Goldberg HJ, Henske EP, El-Chemaly S. The effect of mTOR inhibitors on respiratory infections in lymphangioleiomyomatosis. Eur Respir Rev 2017; 26.
  56. Sebaaly J, Bowers L, Mazur J, et al. Rapid oral desensitization to sirolimus in a patient with lymphangioleiomyomatosis. J Allergy Clin Immunol Pract 2016; 4:352.
  57. Argula RG, Kokosi M, Lo P, et al. A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis. Ann Am Thorac Soc 2016; 13:342.
  58. Schmithorst VJ, Altes TA, Young LR, et al. Automated algorithm for quantifying the extent of cystic change on volumetric chest CT: initial results in Lymphangioleiomyomatosis. AJR Am J Roentgenol 2009; 192:1037.
  59. Dancey JE. Inhibitors of the mammalian target of rapamycin. Expert Opin Investig Drugs 2005; 14:313.
  60. Goldberg HJ, Harari S, Cottin V, et al. Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study. Eur Respir J 2015; 46:783.
  61. Bissler JJ, Kingswood JC, Radzikowska E, et al. Everolimus for renal angiomyolipoma in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis: extension of a randomized controlled trial. Nephrol Dial Transplant 2016; 31:111.
  62. El-Chemaly S, Taveira-Dasilva A, Goldberg HJ, et al. Sirolimus and Autophagy Inhibition in Lymphangioleiomyomatosis: Results of a Phase I Clinical Trial. Chest 2017; 151:1302.
  63. 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. Am J Respir Crit Care Med 2017; 196:1337.
  64. Boehler A, Speich R, Russi EW, Weder W. Lung transplantation for lymphangioleiomyomatosis. N Engl J Med 1996; 335:1275.
  65. Kpodonu J, Massad MG, Chaer RA, et al. The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis. J Heart Lung Transplant 2005; 24:1247.
  66. Pechet TT, Meyers BF, Guthrie TJ, et al. Lung transplantation for lymphangioleiomyomatosis. J Heart Lung Transplant 2004; 23:301.
  67. Maurer JR, Ryu J, Beck G, et al. Lung transplantation in the management of patients with lymphangioleiomyomatosis: baseline data from the NHLBI LAM Registry. J Heart Lung Transplant 2007; 26:1293.
  68. Benden C, Rea F, Behr J, et al. Lung transplantation for lymphangioleiomyomatosis: the European experience. J Heart Lung Transplant 2009; 28:1.
  69. Nine JS, Yousem SA, Paradis IL, et al. Lymphangioleiomyomatosis: recurrence after lung transplantation. J Heart Lung Transplant 1994; 13:714.
  70. Collins J, Müller NL, Kazerooni EA, et al. Lung transplantation for lymphangioleiomyomatosis: role of imaging in the assessment of complications related to the underlying disease. Radiology 1999; 210:325.
  71. Nakagiri T, Shintani Y, Minami M, et al. Lung Transplantation for Lymphangioleiomyomatosis in a Single Japanese Institute, With a Focus on Late-onset Complications. Transplant Proc 2015; 47:1977.
  72. Ussavarungsi K, Hu X, Scott JP, et al. Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis. Respir Med 2015; 109:1354.
  73. Reynaud-Gaubert M, Mornex JF, Mal H, et al. Lung transplantation for lymphangioleiomyomatosis: the French experience. Transplantation 2008; 86:515.
  74. Ando K, Okada Y, Akiba M, et al. Lung Transplantation for Lymphangioleiomyomatosis in Japan. PLoS One 2016; 11:e0146749.
  75. Karbowniczek M, Astrinidis A, Balsara BR, et al. Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism. Am J Respir Crit Care Med 2003; 167:976.
  76. Zaki KS, Aryan Z, Mehta AC, et al. Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation. World J Transplant 2016; 6:249.
  77. Gupta N, Han MK, McCormack FX. Regional Sparing in an Oligemic Lung Segment Supports Hematogenous Spread as a Pathogenic Mechanism in Lymphangioleiomyomatosis. Ann Am Thorac Soc 2015; 12:1247.
  78. Groetzner J, Kur F, Spelsberg F, et al. Airway anastomosis complications in de novo lung transplantation with sirolimus-based immunosuppression. J Heart Lung Transplant 2004; 23:632.
  79. King-Biggs MB, Dunitz JM, Park SJ, et al. Airway anastomotic dehiscence associated with use of sirolimus immediately after lung transplantation. Transplantation 2003; 75:1437.
  80. El-Chemaly S, Goldberg HJ, Glanville AR. Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation? Expert Rev Respir Med 2014; 8:657.
  81. Malouf MA, Hopkins P, Snell G, et al. An investigator-driven study of everolimus in surgical lung biopsy confirmed idiopathic pulmonary fibrosis. Respirology 2011; 16:776.
  82. Egan JJ, Remund KF, Corris P. Sirolimus for lymphangioleiomyomatosis lesions. N Engl J Med 2008; 358:1963.
  83. Glanville AR, Aboyoun C, Klepetko W, et al. Three-year results of an investigator-driven multicenter, international, randomized open-label de novo trial to prevent BOS after lung transplantation. J Heart Lung Transplant 2015; 34:16.
  84. Ito T, Suno M, Sakamoto K, et al. Therapeutic Effect of Sirolimus for Lymphangioleiomyomatosis Remaining in the Abdominopelvic Region After Lung Transplantation: A Case Report. Transplant Proc 2016; 48:271.
  85. Ohara T, Oto T, Miyoshi K, et al. Sirolimus ameliorated post lung transplant chylothorax in lymphangioleiomyomatosis. Ann Thorac Surg 2008; 86:e7.
  86. Almoosa KF, Ryu JH, Mendez J, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest 2006; 129:1274.
  87. Hayashida M, Seyama K, Inoue Y, et al. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology 2007; 12:523.
  88. Young LR, Almoosa KF, Pollock-Barziv S, et al. Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis. Chest 2006; 129:1267.
  89. MacDuff A, Arnold A, Harvey J, BTS Pleural Disease Guideline Group. Management of spontaneous pneumothorax: British Thoracic Society Pleural Disease Guideline 2010. Thorax 2010; 65 Suppl 2:ii18.
  90. Baumann MH, Strange C, Heffner JE, et al. Management of spontaneous pneumothorax: an American College of Chest Physicians Delphi consensus statement. Chest 2001; 119:590.
  91. Hagaman JT, Schauer DP, McCormack FX, Kinder BW. Screening for lymphangioleiomyomatosis by high-resolution computed tomography in young, nonsmoking women presenting with spontaneous pneumothorax is cost-effective. Am J Respir Crit Care Med 2010; 181:1376.
  92. Gupta N, Langenderfer D, McCormack FX, et al. Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective. Ann Am Thorac Soc 2017; 14:17.
  93. Ellender CM, Williams TJ, Gooi J, et al. Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis. Respirol Case Rep 2015; 3:72.
  94. Barrera P, Simons SO, Luijk B, et al. Efficacy of sirolimus therapy for chylous effusions in lymphangioleiomyomatosis. Ann Am Thorac Soc 2013; 10:408.
  95. Rozenberg D, Thenganatt J. Dramatic response to sirolimus in lymphangioleiomyomatosis. Can Respir J 2013; 20:413.
  97. Freitas CS, Baldi BG, Araújo MS, et al. Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations. J Bras Pneumol 2015; 41:275.
  98. Fremont RD, Milstone AP, Light RW, Ninan M. Chylothoraces after lung transplantation for lymphangioleiomyomatosis: review of the literature and utilization of a pleurovenous shunt. J Heart Lung Transplant 2007; 26:953.
  99. Lefrou L, d'Alteroche L, Harchaoui Y, et al. Peritoneovenous shunt after failure of octreotide treatment for chylous ascites in lymphangioleiomyomatosis. Dig Dis Sci 2007; 52:3188.
  100. Paul S, Su S, Edenfield H, et al. Treatment of refractory lymphangioleiomyomatosis-associated chylous effusion with a pleuroperitoneal window and omental flap. J Thorac Cardiovasc Surg 2009; 138:497.
  101. Terzi A, Magnanelli G, Furlan G, Norsa A. [Pulmonary lymphangioleiomyomatosis. Case report]. Minerva Chir 1994; 49:1009.
  102. Basnet A, Shaaban H, Kessler W. A case of recurrent retroperitoneal lymphangioleiomyomatosis treated with progesterone therapy. Urol Ann 2015; 7:127.
  103. Oishi H, Hoshikawa Y, Sado T, et al. A Case of Successful Therapy by Intrapleural Injection of Fibrin Glue for Chylothorax after Lung Transplantation for Lymphangioleiomyomatosis. Ann Thorac Cardiovasc Surg 2017; 23:40.
  104. Mohammadieh AM, Bowler SD, Silverstone EJ, et al. Everolimus treatment of abdominal lymphangioleiomyoma in five women with sporadic lymphangioleiomyomatosis. Med J Aust 2013; 199:121.
  105. Krueger DA, Northrup H, International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 2013; 49:255.
  106. Avila NA, Dwyer AJ, Rabel A, Moss J. Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features. Radiology 2007; 242:277.
  107. Ciftci AO, Sanlialp I, Tanyel FC, Buyukpamukçu N. The association of pulmonary lymphangioleiomyomatosis with renal and hepatic angiomyolipomas in a prepubertal girl: a previously unreported entity. Respiration 2007; 74:335.
  108. Kim NR, Chung MP, Park CK, et al. Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man. Pathol Int 2003; 53:231.
  109. Cottin V, Harari S, Humbert M, et al. Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients. Eur Respir J 2012; 40:630.
  110. Zafar MA, McCormack FX, Rahman S, et al. Pulmonary vascular shunts in exercise-intolerant patients with lymphangioleiomyomatosis. Am J Respir Crit Care Med 2013; 188:1167.
  111. Taveira-DaSilva AM, Jones AM, Julien-Williams PA, et al. Retrospective review of combined sirolimus and simvastatin therapy in lymphangioleiomyomatosis. Chest 2015; 147:180.
  112. Pimenta SP, Baldi BG, Acencio MM, et al. Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade. J Bras Pneumol 2011; 37:424.
  113. Pimenta SP, Baldi BG, Kairalla RA, Carvalho CR. Doxycycline use in patients with lymphangioleiomyomatosis: biomarkers and pulmonary function response. J Bras Pneumol 2013; 39:5.
  114. Chang WY, Cane JL, Kumaran M, et al. A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis. Eur Respir J 2014; 43:1114.
  115. Moses MA, Harper J, Folkman J. Doxycycline treatment for lymphangioleiomyomatosis with urinary monitoring for MMPs. N Engl J Med 2006; 354:2621.
  116. Baldi BG, Ribeiro Carvalho CR. Doxycycline in lymphangioleiomyomatosis: not all questions are answered. Eur Respir J 2014; 43:1536.
  117. Eliasson AH, Phillips YY, Tenholder MF. Treatment of lymphangioleiomyomatosis. A meta-analysis. Chest 1989; 96:1352.
  118. Urban T, Kuttenn F, Gompel A, et al. Pulmonary lymphangiomyomatosis. Follow-up and long-term outcome with antiestrogen therapy; a report of eight cases. Chest 1992; 102:472.
  119. Schiavina M, Contini P, Fabiani A, et al. Efficacy of hormonal manipulation in lymphangioleiomyomatosis. A 20-year-experience in 36 patients. Sarcoidosis Vasc Diffuse Lung Dis 2007; 24:39.
  120. Taveira-DaSilva AM, Stylianou MP, Hedin CJ, et al. Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone. Chest 2004; 126:1867.
  121. Rossi GA, Balbi B, Oddera S, et al. Response to treatment with an analog of the luteinizing-hormone-releasing hormone in a patient with pulmonary lymphangioleiomyomatosis. Am Rev Respir Dis 1991; 143:174.
  122. Banner AS, Carrington CB, Emory WB, et al. Efficacy of oophorectomy in lymphangioleiomyomatosis and benign metastasizing leiomyoma. N Engl J Med 1981; 305:204.
  123. Urban T, Lazor R, Lacronique J, et al. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Medicine (Baltimore) 1999; 78:321.
  124. Johnson SR, Tattersfield AE. Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment. Am J Respir Crit Care Med 1999; 160:628.
  125. Harari S, Cassandro R, Chiodini I, et al. Effect of a gonadotrophin-releasing hormone analogue on lung function in lymphangioleiomyomatosis. Chest 2008; 133:448.
  126. Baldi BG, Freitas CS, Araujo MS, et al. Clinical course and characterisation of lymphangioleiomyomatosis in a Brazilian reference centre. Sarcoidosis Vasc Diffuse Lung Dis 2014; 31:129.
  127. Hayashida M, Yasuo M, Hanaoka M, et al. Reductions in pulmonary function detected in patients with lymphangioleiomyomatosis: An analysis of the Japanese National Research Project on Intractable Diseases database. Respir Investig 2016; 54:193.
  128. Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis. Clinical course in 32 patients. N Engl J Med 1990; 323:1254.
  129. Taveira-DaSilva AM, Steagall WK, Moss J. Lymphangioleiomyomatosis. Cancer Control 2006; 13:276.
  130. Kitaichi M, Nishimura K, Itoh H, Izumi T. Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors. Am J Respir Crit Care Med 1995; 151:527.
  131. Oprescu N, McCormack FX, Byrnes S, Kinder BW. Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry. Lung 2013; 191:35.
  132. Bee J, Bhatt R, McCafferty I, Johnson SR. A 4-year prospective evaluation of protocols to improve clinical outcomes for patients with lymphangioleiomyomatosis in a national clinical centre. Thorax 2015; 70:1202.
  133. Johnson SR, Whale CI, Hubbard RB, et al. Survival and disease progression in UK patients with lymphangioleiomyomatosis. Thorax 2004; 59:800.
  134. Reid JK, Rees H, Cockcroft D. Long term survival in a patient with pulmonary lymphangioleiomyomatosis. Can Respir J 2002; 9:342.
  135. Yigla M, Bentur L, Ben Izhak O, Rubin AH. Pulmonary lymphangioleiomyomatosis: prolonged survival despite multiple pregnancies and no hormonal intervention. Respirology 1996; 1:213.
  136. Matsui K, Beasley MB, Nelson WK, et al. Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score. Am J Surg Pathol 2001; 25:479.
  137. Taveira-DaSilva AM, Jones AM, Julien-Williams P, et al. Severity and outcome of cystic lung disease in women with tuberous sclerosis complex. Eur Respir J 2015; 45:171.
  138. Taveira-DaSilva AM, Steagall WK, Rabel A, et al. Reversible airflow obstruction in lymphangioleiomyomatosis. Chest 2009; 136:1596.
  139. Crausman RS, Lynch DA, Mortenson RL, et al. Quantitative CT predicts the severity of physiologic dysfunction in patients with lymphangioleiomyomatosis. Chest 1996; 109:131.
  140. Lo P, Brown MS, Kim H, et al. Cyst-based measurements for assessing lymphangioleiomyomatosis in computed tomography. Med Phys 2015; 42:2287.
  141. Baldi BG, Araujo MS, Freitas CS, et al. Evaluation of the extent of pulmonary cysts and their association with functional variables and serum markers in lymphangioleiomyomatosis (LAM). Lung 2014; 192:967.