Official American Thoracic Society/Japanese Respiratory Society clinical practice guidelines: lymphangioleiomyomatosis diagnosis and management
FX McCormack, N Gupta, GR Finlay… - American journal of …, 2016 - atsjournals.org
FX McCormack, N Gupta, GR Finlay, LR Young, AM Taveira-DaSilva, CG Glasgow…
American journal of respiratory and critical care medicine, 2016•atsjournals.orgBackground: 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 …
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 …
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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