title: The St. George's Respiratory Questionnaire in lymphangioleiomyomatosis.
abstract: OBJECTIVE
  To examine the correlation between the health-related quality of life measured by the St. George's Respiratory Questionnaire (SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis (LAM).
  
  
  METHODS
  This study retrospectively analyzed the SGRQ scores and other measures (the Borg scale of breathlessness at rest, 6-minute walking distance, blood oxygen levels, and pulmonary function) of patients diagnosed and confirmed with LAM. Altogether 38 patients between June 2007 and November 2009 were included.
  
  
  RESULTS
  The mean values of the SGRQ three components (symptoms, activity, and impacts) and total scores in the LAM patients were 46.95 +/- 28.90, 58.47 +/- 25.41, 47.89 +/- 29.66, and 51.11 +/- 26.35, respectively. The SGRQ total or component scores were correlated well with the Borg scale of breathlessness, 6-minute walking distance, partial pressure of oxygen in arterial blood, spirometry and diffusion capacity of lung. There were poor correlations between SGRQ score and residual volume or total lung capacity. In our preliminary observation, sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment (n = 6).
  
  
  CONCLUSIONS
  The SGRQ score in LAM is correlated well with physiological measures (Borg scale of breathlessness, 6-minute walking distance, blood oxygen levels, and pulmonary function tests). The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM. Treatment with sirolimus, an inhibitor of mammalian target of rapamycin, may improve the quality of life and patient's perception of breathlessness in LAM.
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China. kaifeng.xu@gmail.com
authors.forename: Kai-Feng
authors.initials: KF
authors.lastname: Xu
authors.forename: Lan
authors.initials: L
authors.lastname: Wang
authors.forename: Xin-Lun
authors.initials: XL
authors.lastname: Tian
authors.forename: Yao-Song
authors.initials: YS
authors.lastname: Gui
authors.forename: Min
authors.initials: M
authors.lastname: Peng
authors.forename: Bai-Qiang
authors.initials: BQ
authors.lastname: Cai
authors.forename: Yuan-Jue
authors.initials: YJ
authors.lastname: Zhu
mesh_terms: D000328:Adult; D005541:Forced Expiratory Volume; D006801:Humans; D018192:Lymphangioleiomyomatosis / Q000503:physiopathology / Q000523:psychology*; D008875:Middle Aged; D011788:Quality of Life; D012115:Residual Volume; D011795:Surveys and Questionnaires; D014797:Vital Capacity