Spontaneous pneumothoraces during pregnancy in patients with lymphangioleiomyomatosis

Respir Investig. 2023 Sep;61(5):632-635. doi: 10.1016/j.resinv.2023.06.006. Epub 2023 Jul 12.

Abstract

Patients with lymphangioleiomyomatosis (LAM) are prone to developing spontaneous pneumothoraces (SPs). We aimed to characterize the burden of SPs during pregnancy in LAM, using a web-based survey. Among the 50 respondents, 12 (24%) had never been pregnant and 38 (76%) were pregnant at least once, resulting in a total of 80 pregnancies. Respiratory symptoms during pregnancy led to the diagnosis of LAM in 34% of (13/38) patients, with SPs being the presenting manifestation in most of these patients (10/13, 77%). Eleven of the 38 pregnant patients (29%) experienced at least one SP during pregnancy. The majority of the SPs (∼60%) occurred during the second trimester. Our study provides valuable insights regarding the risk, burden, and timing of pregnancy-related SPs in patients with LAM that would be useful for the LAM community.

Keywords: Estrogen; LAM; Pleurodesis; TSC; mTOR.

MeSH terms

  • Female
  • Humans
  • Lung Neoplasms* / diagnosis
  • Lymphangioleiomyomatosis* / complications
  • Lymphangioleiomyomatosis* / diagnosis
  • Pneumothorax* / epidemiology
  • Pneumothorax* / etiology
  • Pregnancy