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 46.9528.90

58.4725.41

47.8929.66

and 51.1126.35

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.