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Guidelines Developed for Lymphangioleiomyomatosis

Evidence-based guidelines include tx with sirolimus, vascular endothelial growth factor D testing


FRIDAY, Sept. 16, 2016 (HealthDay News) -- Evidence-based guidelines have been developed for the diagnosis and treatment of lymphangioleiomyomatosis (LAM). The clinical practice guidelines were published online Sept. 15 in the American Journal of Respiratory and Critical Care Medicine.

Francis X. McCormack, M.D., from the University of Cincinnati, and colleagues conducted systematic reviews of evidence relating to diagnosis and treatment of LAM. A multidisciplinary panel summarized and discussed the evidence. Recommendations were formulated, written, and graded.

The authors note that recommendations were formulated for or against each specific intervention after considering the estimated effects, balance of benefits and harms and burden of treatment, consequences of treatment, patient values and preferences, cost, and feasibility. Based on evidence of varying quality, treatment with sirolimus was recommended rather than observation for patients with LAM with abnormal/declining lung function; treatment with sirolimus was recommended before invasive management for selected patients with LAM with problematic chylous effusions. Based on low-quality evidence, doxycycline was not recommended as treatment for LAM. Hormonal therapy should not be used as a treatment for LAM. Vascular endothelial growth factor D testing is recommended for establishing the diagnosis of LAM before considering diagnostic lung biopsy.

"The guidelines cannot take into account all of the complexities that clinicians face when making treatment decisions for patients with a rare lung disease," a coauthor said in a statement.

Several authors disclosed financial ties to the pharmaceutical industry.

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