Decline in lung function in lymphangioleiomyomatosis - Relation to menopause and progesterone treatment

The progression of lymphangioleiomyomatosis, a rare lung disease in women, is thought to be influenced by hormonal factors. We studied the rate of dec line in FEV1 and carbon monoxide transfer factor (TLCO) in a national cohor t of patients with lymphangioleiomyomatosis in the United Kingdom and its r elation to two factors that might influence the disease, menopausal status and progesterone treatment. We used retrospective data from hospital notes, and of the 50 patients identified 43 had suitable lung function data spann ing at least 3 mo. Mean (SD) annual decline in FEV1 was 118 (142) mi for al l patients, and these figures changed little when only data spanning at lea st 2 and 3 yr were analyzed. There was considerable variation in the rate o f decline between subjects, however, and although it tended to be less amon g postmenopausal women and those receiving progesterone, patient numbers we re smaller and the findings were not significant. There was a significant r eduction in decline in TLCO in premenopausal patients receiving progesteron e and in both FEV1 and TLCO after starting progesterone in six patients who had data before and after starting treatment. This study documents the rap id decline in lung function in lymphangioleiomyomatosis, confirms the wide variation between patients, and provides some support for the suggestion th at disease progression may be reduced by progesterone. The data provide a b asis for designing prospective studies of treatment for lymphangioleiomyoma tosis.