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. Henske & 12 others John 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

67 Citations (Scopus)

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

Fingerprint

Lymphangioleiomyomatosis
Practice Guidelines
Thorax
Vascular Endothelial Growth Factor D
Therapeutics
Patient Preference
Doxycycline
Sirolimus
Guidelines
Costs and Cost Analysis

All Science Journal Classification (ASJC) codes

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Cite this

Official American thoracic society/Japanese respiratory society clinical practice guidelines : Lymphangioleiomyomatosis diagnosis and management. / McCormack, Francis X.; Gupta, Nishant; Finlay, Geraldine R.; Young, Lisa R.; Taveira-Da Silva, Angelo M.; Glasgow, Connie G.; Steagall, Wendy K.; Johnson, Simon R.; Sahn, Steven A.; Ryu, Jay H.; Strange, Charlie; Seyama, Kuniaki; Sullivan, Eugene J.; Kotloff, Robert M.; Downey, Gregory P.; Chapman, Jeffrey T.; Han, Meilan K.; D'Armiento, Jeanine M.; Inoue, Yoshikazu; Henske, Elizabeth P.; Bissler, John; Colby, Thomas V.; Kinder, Brent W.; Wikenheiser-Brokamp, Kathryn A.; Brown, Kevin K.; Cordier, Jean F.; Meyer, Cristopher; Cottin, Vincent; Brozek, Jan L.; Smith, Karen; Wilson, Kevin C.; Moss, Joel.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 194, No. 6, 15.09.2016, p. 748-761.

Research output: Contribution to journalArticle

McCormack, FX, Gupta, N, Finlay, GR, Young, LR, Taveira-Da Silva, AM, Glasgow, CG, Steagall, WK, Johnson, SR, Sahn, SA, Ryu, JH, Strange, C, Seyama, K, Sullivan, EJ, Kotloff, RM, Downey, GP, Chapman, JT, Han, MK, D'Armiento, JM, Inoue, Y, Henske, EP, Bissler, J, Colby, TV, Kinder, BW, Wikenheiser-Brokamp, KA, Brown, KK, Cordier, JF, Meyer, C, Cottin, V, Brozek, JL, Smith, K, Wilson, KC & 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, vol. 194, no. 6, pp. 748-761. https://doi.org/10.1164/rccm.201607-1384ST
McCormack, Francis X. ; Gupta, Nishant ; Finlay, Geraldine R. ; Young, Lisa R. ; Taveira-Da Silva, Angelo M. ; Glasgow, Connie G. ; Steagall, Wendy K. ; Johnson, Simon R. ; Sahn, Steven A. ; Ryu, Jay H. ; Strange, Charlie ; Seyama, Kuniaki ; Sullivan, Eugene J. ; Kotloff, Robert M. ; Downey, Gregory P. ; Chapman, Jeffrey T. ; Han, Meilan K. ; D'Armiento, Jeanine M. ; Inoue, Yoshikazu ; Henske, Elizabeth P. ; Bissler, John ; Colby, Thomas V. ; Kinder, Brent W. ; Wikenheiser-Brokamp, Kathryn A. ; Brown, Kevin K. ; Cordier, Jean F. ; Meyer, Cristopher ; Cottin, Vincent ; Brozek, Jan L. ; Smith, Karen ; Wilson, Kevin C. ; Moss, Joel. / Official American thoracic society/Japanese respiratory society clinical practice guidelines : Lymphangioleiomyomatosis diagnosis and management. In: American Journal of Respiratory and Critical Care Medicine. 2016 ; Vol. 194, No. 6. pp. 748-761.
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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.",
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T2 - Lymphangioleiomyomatosis diagnosis and management

AU - McCormack, Francis X.

AU - Gupta, Nishant

AU - Finlay, Geraldine R.

AU - Young, Lisa R.

AU - Taveira-Da Silva, Angelo M.

AU - Glasgow, Connie G.

AU - Steagall, Wendy K.

AU - Johnson, Simon R.

AU - Sahn, Steven A.

AU - Ryu, Jay H.

AU - Strange, Charlie

AU - Seyama, Kuniaki

AU - Sullivan, Eugene J.

AU - Kotloff, Robert M.

AU - Downey, Gregory P.

AU - Chapman, Jeffrey T.

AU - Han, Meilan K.

AU - D'Armiento, Jeanine M.

AU - Inoue, Yoshikazu

AU - Henske, Elizabeth P.

AU - Bissler, John

AU - Colby, Thomas V.

AU - Kinder, Brent W.

AU - Wikenheiser-Brokamp, Kathryn A.

AU - Brown, Kevin K.

AU - Cordier, Jean F.

AU - Meyer, Cristopher

AU - Cottin, Vincent

AU - Brozek, Jan L.

AU - Smith, Karen

AU - Wilson, Kevin C.

AU - Moss, Joel

PY - 2016/9/15

Y1 - 2016/9/15

N2 - 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.

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