Background: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to

Lymphangioleiomyomatosis (LAM) is an uncommon disease presented as diffuse thin-walled cystic changes in the lung. The main differential diagnoses include pulmonary Langerhans’ histiocytosis (PLCH), Birt-Hogg-Dubé syndrome (BHD), lymphoid interstitial

Lymphangioleiomyomatosis (LAM) is a primary disease of the lung parenchyma typified by abnormal growth of atypical smooth muscle cells in the lung vasculature, lymphatics, and alveoli that leads to the formation of multiple cysts in the lungs bilaterally