Table 1.

Summary of the Recommendations Provided in This Guideline

Context	Recommendation	Strength of Recommendation	Confidence in Estimates of Effect
Treatment with mTOR inhibitors	For patients with LAM with abnormal/declining lung function, we recommend treatment with sirolimus rather than observation.	Strong	Moderate
	For selected patients with LAM with problematic chylous effusions, we suggest treatment with sirolimus before invasive management.	Conditional	Very low
Treatment with doxycycline	We suggest NOT using doxycycline as treatment for LAM.	Conditional	Low
Treatment with hormonal therapy	We suggest NOT using hormonal therapy as treatment for LAM. (“Hormonal therapy” includes the progestins, GnRH agonists, selective estrogen receptor modulators like tamoxifen, and oophorectomy.)	Conditional	Very low
VEGF-D as a diagnostic test	For patients whose CT scan shows cystic abnormalities characteristic of LAM but have no confirmatory clinical or extrapulmonary radiologic features of LAM, we recommend VEGF-D testing before consideration of proceeding to diagnostic lung biopsy. (“Confirmatory features of LAM” include tuberous sclerosis complex, angiomyolipomas, chylous pleural effusions or ascites, and cystic lymphangioleiomyomas.)	Strong	Moderate

Definition of abbreviations: CT = computed tomography; GnRH = gonadotrophin-releasing hormone; LAM = lymphangioleiomyomatosis; mTOR = mechanistic target of rapamycin; VEGF-D = vascular endothelial growth factor D.