Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts

Thorax. 2020 Oct;75(10):904-907. doi: 10.1136/thoraxjnl-2020-214987. Epub 2020 Aug 11.

Abstract

Pregnancy in women with lymphangioleiomyomatosis (LAM) has been associated with increased complications and worsening lung function although objective data to advise patients are not available. We assessed lung function and CT scans before and after pregnancy in 16 women with LAM. During the pregnancy, pneumothorax was frequent and mean forced expiratory volume in 1 s (FEV1) fell from 77%±19% prepregnancy to 64%±25% predicted and DLCO from 66±26 to 57±26 (both p<0.01). After pregnancy, rates of FEV1 decline were high and 10 patients required sirolimus. Women with LAM, especially with moderate or advanced disease should be counselled regarding adverse events and loss of lung function during the pregnancy.

Keywords: rare lung diseases.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • Adult
  • Cohort Studies
  • Female
  • Forced Expiratory Volume
  • Humans
  • Lung Neoplasms / complications
  • Lung Neoplasms / physiopathology*
  • Lung Neoplasms / therapy*
  • Lymphangioleiomyomatosis / complications
  • Lymphangioleiomyomatosis / physiopathology*
  • Lymphangioleiomyomatosis / therapy*
  • Pneumothorax / etiology
  • Pregnancy
  • Pregnancy Complications, Neoplastic / etiology
  • Pregnancy Complications, Neoplastic / physiopathology*
  • Pregnancy Complications, Neoplastic / therapy*
  • Pregnancy Outcome
  • Vital Capacity
  • Young Adult