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Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use
  1. Jan Johnson,
  2. Simon R Johnson
  1. Division of Respiratory Medicine and NIHR Biomedical Research Centre and National Centre for LAM, Nottingham Unioversity NHS Trust, Nottingham UK, University of Nottingham, Nottingham, UK
  1. Correspondence to Professor Simon R Johnson, Respiratory Medicine, University of Nottingham, Nottingham, NG7 2RD, UK; simon.johnson{at}


Lymphangioleiomyomatosis can be associated with reversible airflow obstruction and although no guidelines around reversibility testing or inhaled therapy exist, many patients receive bronchodilators and inhaled corticosteroids. To better identify those who may benefit, we examined bronchodilator reversibility and inhaled therapy in a national cohort of 213 subjects. 20% of those tested had airway reversibility by standard criteria. 55% of patients used 13 different combinations of bronchodilators and inhaled corticosteroids. Increasing inhaler classes were associated with reversibility and more rapid FEV1 decline. Reversibility testing should be performed in all patients and inhaled therapy should be formally studied.

  • rare lung diseases

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  • Contributors SJ devised the study, saw all patients, entered the study data, analysed the results and wrote the paper. JJ analysed the data and wrote the paper.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval The East Midlands Research Ethics Committee approved the study.

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

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