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A new lymphangioleiomyomatosis clinical practice guideline

American Thoracic Society News Jul 20, 2017

In the July Annals of the American Thoracic Society, the ATS and Japanese Respiratory Society published the following recommendations addressing the use of vascular endothelial growth factor D (VEGF–D) as a diagnostic test and the therapeutic role of sirolimus, doxycycline and hormonal manipulation in patients with lymphangioleiomyomatosis:
  • For selected patients with LAM with problematic chylous effusions, we suggest treatment with sirolimus before invasive management.
  • We suggest NOT using doxycycline as treatment for LAM.
  • We suggest NOT using hormonal therapy as treatment for LAM.
  • For patients whose computed tomography scan shows cystic abnormalities characteristic of LAM, but who have no other confirmatory clinical or extrapulmonary radiologic features of LAM, we recommend vascular endothelial growth factor D testing to establish the diagnosis of LAM before consideration of proceeding to diagnostic lung biopsy.
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