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Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts
  1. Angelo M Taveira-DaSilva1,
  2. Simon R Johnson2,
  3. Patricia Julien-Williams1,
  4. Jan Johnson3,
  5. Mario Stylianou4,
  6. Joel Moss5
  1. 1 Cardiovascular and Pulmonary Branch/NHLBI, NIH, Bethesda, Maryland, USA
  2. 2 Respiratory Medicine, University of Nottingham, NIHR Biomedical Research Centre, Nottingham Biodiscovery Institute and National Centre for LAM, Nottingham, UK
  3. 3 Respiratory Medicine, University of Nottingham Faculty of Medicine and Health Sciences, Nottingham, Nottingham, UK
  4. 4 National Heart, Lung, and Blood Institute, Office of Biostatistics Research, National Institutes of Health, Bethesda, Maryland, USA
  5. 5 Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Hea, Bethesda, Maryland, Maryland, USA
  1. Correspondence to Professor Simon R Johnson, Respiratory Medicine, University of Nottingham, Nottingham NG7 2RD, UK; simon.johnson{at}nottingham.ac.uk

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.

  • rare lung diseases
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Footnotes

  • AMT-D and SRJ contributed equally.

  • Contributors AMT-D, SRJ and JM are responsible for study design, data analysis and writing the manuscript. JJ and PJ-W collected and reviewed clinical data. MS performed the statistical analysis.

  • Funding This study was supported by the Intramural Research Program, National Institutes of Health, National Heart, Lung, and Blood Institute, the Nottingham Biomedical Research Centre, Nottingham Molecular Pathology Node and National Centre for LAM.

  • Competing interests SRJ received grant funding from the NIHR-RDTRC for the current work.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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