TY - JOUR
T1 - Official American thoracic society/Japanese respiratory society clinical practice guidelines
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 J.
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
N1 - Publisher Copyright:
© Copyright 2016 by the American Thoracic Society.
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.
AB - 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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U2 - 10.1164/rccm.201607-1384ST
DO - 10.1164/rccm.201607-1384ST
M3 - Article
C2 - 27628078
AN - SCOPUS:84988961785
SN - 1073-449X
VL - 194
SP - 748
EP - 761
JO - American journal of respiratory and critical care medicine
JF - American journal of respiratory and critical care medicine
IS - 6
ER -