Lymphangioleiomyomatosis Guidelines

Updated: Dec 22, 2019
  • Author: Joel Moss, MD, PhD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
  • Print

Guidelines Summary

Guidelines for the diagnosis and management of lymphangioleiomyomatosis (LAM) have been issued by the following organizations:

  • European Respiratory Society (ERS)
  • American Thoracic Society (ATS) and Japanese Respiratory Society (JRS)

The European Respiratory Society's comprehensive guidelines were published in 2010. However, research advances have identified an effective treatment and a useful diagnostic biomarker since publication. In 2016, the American Thoracic Society (ATS) and Japanese Respiratory Society (JRS) released joint clinical practice guidelines which were endorsed by the LAM Foundation. Key recommendations based on evidence of varying quality include the following [6] :

  • Vascular endothelial growth factor D testing should be used to establish the diagnosis of LAM before considering lung biopsy in patients with cystic abnormalities on high-resolution computed tomography (HRCT) characteristic of LAM, but no other confirmatory clinical features or extrapulmonary radiologic features of LAM.
  • For patients with LAM with abnormal/declining lung function, treatment with sirolimus rather than observation is recommended.
  • For selected patients with LAM with problematic chylous effusions, treatment with sirolimus before invasive management is recommended.
  • Doxycycline and hormonal therapy should not be used for thetreatment of LAM.