Recommendations developed for lymphangioleiomyomatosis

(HealthDay)—In a Clinical Practice Guideline published in the American Journal of Respiratory and Critical Care Medicine, evidence-based recommendations are presented for the diagnosis and management of lymphangioleiomyomatosis (LAM).

Francis X. McCormack, M.D., from the University of Cincinnati, and colleagues performed systematic reviews to summarize the evidence relating to diagnosis and treatment of LAM. Evidence-based recommendations were formulated, written, and graded.

The authors considered the panel's confidence in the estimated effects, the balance of benefits versus harms and burdens of treatment, patient values and preferences, cost, and feasibility, and formulated recommendations for or against specific interventions. Recommendations included treatment with sirolimus rather than observation for patients with LAM with abnormal/declining lung function, and treatment with sirolimus before invasive management for selected patients with LAM with problematic chylous effusions. Vascular endothelial growth factor D testing was recommended to establish LAM diagnosis before consideration of diagnostic lung biopsy. Doxycycline and hormonal therapy were not recommended for LAM treatment.

"Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided," the authors write. "Frequent reassessment and updating will be needed."

Several authors disclosed financial ties to the biopharmaceutical industry.