• 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, and ATS/JRS Committee on Lymphangioleiomyomatosis.
• Am. J. Respir. Crit. Care Med. 2016 Sep 15; 194 (6): 748-61.

BackgroundLymphangioleiomyomatosis (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.MethodsSystematic 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.ResultsAfter 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.ConclusionsEvidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

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