Official American thoracic society/Japanese respiratory society clinical practice guidelines: Lymphangioleiomyomatosis diagnosis and management

Francis X. McCormack, Nishant Gupta, Geraldine R. Finlay, Lisa R. Young, Angelo M. Taveira-Da Silva, 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. HenskeJohn 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

Research output: Contribution to journalArticle

94 Scopus citations

Abstract

Background: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM. Methods: Systematic reviews were performed to summarize evidence pertinent to our questions. The evidence was summarized and discussed by a multidisciplinary panel. Evidence-based recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach. Results: After considering the panel's confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were formulated for or against specific interventions. These included recommendations for sirolimus treatment and vascular endothelial growth factor D testing and recommendations against doxycycline and hormonal therapy. Conclusions: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

Original languageEnglish (US)
Pages (from-to)748-761
Number of pages14
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume194
Issue number6
DOIs
StatePublished - Sep 15 2016

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ASJC Scopus subject areas

  • Medicine(all)
  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Cite this

McCormack, F. X., Gupta, N., Finlay, G. R., Young, L. R., Taveira-Da Silva, A. M., Glasgow, C. G., Steagall, W. K., Johnson, S. R., Sahn, S. A., Ryu, J. H., Strange, C., Seyama, K., Sullivan, E. J., Kotloff, R. M., Downey, G. P., Chapman, J. T., Han, M. K., D'Armiento, J. M., Inoue, Y., ... Moss, J. (2016). Official American thoracic society/Japanese respiratory society clinical practice guidelines: Lymphangioleiomyomatosis diagnosis and management. American Journal of Respiratory and Critical Care Medicine, 194(6), 748-761. https://doi.org/10.1164/rccm.201607-1384ST