The American Thoracic Society (ATS) and the Japanese Respiratory Society (JRS) have published new clinical practice guidelines for the diagnosis and management of lymphangioleiomyomatosis (LAM), a rare lung disease that primarily affects women of child-bearing age and is associated with tuberous sclerosis. Read more about LAM in our information page on TSC and the lungs.
The guidelines in a summary:
- Patients with abnormal or declining lung function should be treated with sirolimus as opposed to observation.
- Patients with problematic chylous effusions should be treated with sirolimus prior to invasive management.
- Doxycyline should not be used as treatment.
- Hormonal therapy should not be used as treatment.
- Patients with LAM–like cystic abnormalities, but no other present characteristics of LAM, should be examined through vascular endothelial growth factor D (VEGF-D) testing first rather than lung biopsy to diagnosis LAM.
If you have any questions about this or want help to find a doctor experienced in LAM, please contact the TSC Information Service on 1300 733 435.
For more information, see:
- Summary article on Science Daily
- ATS Publication: Francis X. McCormack, Nishant Gupta, Geraldine R. Finlay, Lisa R. Young, Angelo M. Taveira-DaSilva, Connie G. Glasgow, Wendy K. Steagall, Simon R. Johnson, Steven A. Sahn, Jay H. Ryu, Charlie Strange, Kuniaki Seyama, Eugene J. Sullivan, Robert M. Kotloff, Gregory P. Downey, Jeffrey T. Chapman, MeiLan K. Han, Jeanine M. D’Armiento, Yoshikazu Inoue, Elizabeth P. Henske, John J. Bissler, Thomas V. Colby, Brent W. Kinder, Kathryn A. Wikenheiser-Brokamp, Kevin K. Brown, Jean F. Cordier, Cristopher Meyer, Vincent Cottin, Jan L. Brozek, Karen Smith, Kevin C. Wilson, Joel Moss. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American Journal of Respiratory and Critical Care Medicine, 2016; 194 (6): 748 DOI: 10.1164/rccm.201607-1384ST