Lymphangioleiomyomatosis Treatment & Management

  • Author: Joel Moss, MD, PhD; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Oct 8, 2010
 

Medical Care

  • General care for lymphangioleiomyomatosis (LAM)
    • Pleural effusions - Chemical pleurodesis; surgical obliteration of the pleural space; medium-chain triglyceride (MCT [not a component of chyle]), lipid-free diet to reduce chyle flow (utility unknown)
    • Ascites - Paracentesis, MCT diet (utility unknown)
    • Pulmonary dysfunction - General pulmonary care (eg, vaccines), bronchodilators (+/- benefit), supplemental oxygen, pulmonary rehabilitation
    • Lung transplantation[6]
  • Hormonal manipulation
    • Medroxyprogesterone - Utility not known, recent case series does not support use
    • Gonadotropin-releasing hormone agonists - Utility not known, few case reports support use
    • Tamoxifen not recommended
    • Oophorectomy not thought to be effective based on recent case series
  • New experimental therapies
    • Rapamycin-initial trials in AML, now being investigated as a therapy in pulmonary LAM
    • Doxycycline-anti-angiogenic, antibiotic and matrix effects
    • Octreotide
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Surgical Care

  • Management of recurrent pneumothoraces or pleural effusions may require surgical intervention; in addition, patients with AML can develop complications (eg, hemorrhage), requiring intervention.
  • Consider lung transplantation for patients with end-stage pulmonary disease.
  • The Medscape Transplantation Specialty Center may be helpful.
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Consultations

  • Pulmonologist
    • Pulmonologist helps establish the diagnosis and monitor pulmonary function.
    • A pulmonologist can address issues such as vaccinations, oxygen therapy, and pulmonary rehabilitation.
    • Depending on disease severity, referral to a transplant center may be beneficial. Consider patient referral to a center with an interest in LAM.
  • Endocrinologist and/or obstetrician-gynecologist
    • These specialists may help to address the hormonal issues involved, particularly when considering hormonal manipulation.
    • An endocrinologist can assist with prophylaxis and treatment of osteoporosis in patients in whom exogenous ER is contraindicated.
  • A urologist may assist with renal AML management.
  • A dietitian may help to consult patients on MCT diets for chylous ascites or pleural effusions.
  • Consider referral to specialist center.
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Diet

  • Most patients with LAM do not have special dietary requirements; however, if a patient is on therapies to lower ER or is postmenopausal and not on ER replacement therapy, address other cardiac risk factors (eg, cholesterol levels).
  • Some patients with chylous effusions or ascites may try an MCT diet (see Medical Care).
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Activity

  • Although the literature is sparse with regard to activities that can cause barotrauma in patients with LAM, theoretical concern exists regarding certain activities such as flying, particularly in patients with LAM and a history of prior pneumothorax.
  • Reports show pregnancy exacerbating the disease; therefore, specialists generally recommend that patients with LAM avoid becoming pregnant. However, some patients with mild disease have had a normal pregnancy with little deterioration in lung function.
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Contributor Information and Disclosures
Author

Joel Moss, MD, PhD  Deputy Chief, Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health

Joel Moss, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Society for Biochemistry and Molecular Biology, American Society for Clinical Investigation, American Thoracic Society, Association of American Physicians, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

John A Kelly, MB, BCh, MD  Assistant Professor of Medicine and Micro-Immunology, Dartmouth Medical School; Staff Pulmonologist, White River Junction Veterans Affairs Medical Center

John A Kelly, MB, BCh, MD is a member of the following medical societies: American Association of Immunologists, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Ryland P Byrd Jr, MD  Professor, Department of Internal Medicine, Division of Pulmonary Medicine and Critical Care Medicine, Program Director of Pulmonary Diseases and Critical Care Medicine Fellowship, James H Quillen College of Medicine, East Tennessee State University; Medical Director of Respiratory Therapy, James H Quillen Veterans Affairs Medical Center

Ryland P Byrd Jr, MD is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Harold L Manning, MD  Associate Professor, Departments of Medicine, Anesthesiology and Physiology, Section of Pulmonary and Critical Care Medicine, Dartmouth Medical School

Harold L Manning, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

References

  1. Carsillo T, Astrinidis A, Henske EP. Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. Proc Natl Acad Sci U S A. May 23 2000;97(11):6085-90. [Medline].

  2. Smolarek TA, Wessner LL, McCormack FX, Mylet JC, Menon AG, Henske EP. Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis. Am J Hum Genet. Apr 1998;62(4):810-5. [Medline].

  3. Young LR, Vandyke R, Gulleman PM, Inoue Y, Brown KK, Schmidt LS, et al. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest. Sep 2010;138(3):674-81. [Medline]. [Full Text].

  4. Glasgow CG, Avila NA, Lin JP, Stylianou MP, Moss J. Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement. Chest. May 2009;135(5):1293-300. [Medline]. [Full Text].

  5. Bonetti F, Chiodera PL, Pea M, Martignoni G, Bosi F, Zamboni G, et al. Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis. Am J Surg Pathol. Nov 1993;17(11):1092-102. [Medline].

  6. Boehler A, Speich R, Russi EW, Weder W. Lung transplantation for lymphangioleiomyomatosis. N Engl J Med. Oct 24 1996;335(17):1275-80. [Medline].

  7. 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. Feb 1995;151(2 Pt 1):527-33. [Medline].

  8. Adema GJ, de Boer AJ, Vogel AM, Loenen WA, Figdor CG. Molecular characterization of the melanocyte lineage-specific antigen gp100. J Biol Chem. Aug 5 1994;269(31):20126-33. [Medline].

  9. Bonetti F, Pea M, Martignoni G, Zamboni G, Iuzzolino P. Cellular heterogeneity in lymphangiomyomatosis of the lung. Hum Pathol. Jul 1991;22(7):727-8. [Medline].

  10. Corrin B, Liebow AA, Friedman PJ. Pulmonary lymphangiomyomatosis. A review. Am J Pathol. May 1975;79(2):348-82. [Medline].

  11. Eliasson AH, Phillips YY, Tenholder MF. Treatment of lymphangioleiomyomatosis. A meta-analysis. Chest. Dec 1989;96(6):1352-5. [Medline].

  12. Kalassian KG, Doyle R, Kao P, Ruoss S, Raffin TA. Lymphangioleiomyomatosis: new insights. Am J Respir Crit Care Med. Apr 1997;155(4):1183-6. [Medline].

  13. Moss J. LAM and Other Diseases Characterized by Smooth Muscle Proliferation. ed. New York, NY: Marcel Decker; 1999.

  14. Moss J, DeCastro R, Patronas NJ, Taveira-DaSilva A. Meningiomas in lymphangioleiomyomatosis. JAMA. Oct 17 2001;286(15):1879-81. [Medline].

  15. Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. Jan 1 2006;173(1):105-11. [Medline].

  16. Steagall WK, Glasgow CG, Hathaway OM, Avila NA, Taveira-Dasilva AM, Rabel A, et al. Genetic and Morphologic Determinants of Pneumothorax in Lymphangioleiomyomatosis. Am J Physiol Lung Cell Mol Physiol. Jul 6 2007;[Medline].

  17. Steagall WK, Taveira-DaSilva AM, Moss J. Clinical and molecular insights into lymphangioleiomyomatosis. Sarcoidosis Vasc Diffuse Lung Dis. Dec 2005;22 Suppl 1:S49-66. [Medline].

  18. Taveira-DaSilva AM, Stylianou MP, Hedin CJ, Hathaway O, Moss J. Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone. Chest. Dec 2004;126(6):1867-74. [Medline].

  19. Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis. Clinical course in 32 patients. N Engl J Med. Nov 1 1990;323(18):1254-60. [Medline].

  20. Urban T, Kuttenn F, Gompel A, Marsac J, Lacronique J. Pulmonary lymphangiomyomatosis. Follow-up and long-term outcome with antiestrogen therapy; a report of eight cases. Chest. Aug 1992;102(2):472-6. [Medline].

 
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