Relevant aspects of lymphangioleiomyomatosis:

  • almost exclusively affects women of child-bearing age
  • it is a multi-system disorder and can affect many organs
  • recurrent pneumothorax can occur in up to 80% of cases
  • in the lungs it manifests as multiple thin-walled cysts throughout the lungs (usually with a uniform distribution)
  • for pulmonary manifestations, the primary differential to be considered is Langerhans cell histiocytosis (LCH) which tends to happen in children and young adults with a history of heavy cigarette smoking