Abstract
Lymphangioleiomyomatosis (LAM) is characterised by lung cysts and airflow obstruction. Matrix metalloproteinases have been implicated in lung destruction in LAM. We performed a randomised, double-blind trial, comparing the matrix metalloproteinases inhibitor doxycycline with placebo on the progression of LAM.
23 females with LAM were randomised to doxycycline 100 mg daily for 3 months followed by 200 mg daily for 21 months, or matched placebo. Lung function, exercise capacity, quality of life and matrix metalloproteinases levels were measured.
21 patients completed 6 months of treatment, 17 completed 1 year of treatment and 15 completed 2 years of treatment. Eight withdrew from the trial due, four due to a pneumothorax and four because of other reasons. The mean±sd decline in FEV1, the primary endpoint, did not differ between the groups being -90±154 mL·year−1 in the placebo group and -123±246 mL·year−1 in the doxycycline group (difference -32.5, 95% CI -213–148; p=0.35). Doxycycline had no effect upon vital capacity, gas transfer, shuttle walk distance or quality of life. Urine matrix metalloproteinases-9 measurements were lower with doxycycline treatment (p=0.03).
Although with limited numbers we cannot completely exclude an effect of doxycycline, the lack of effect on any outcome makes it unlikely that doxycycline has a useful effect in LAM.
Abstract
Doxycycline does not prevent progression of lung disease in LAM http://ow.ly/tiMqo
Footnotes
For editorial comments see page 967.
This article has supplementary material available from www.erj.ersjournals.com
Support statement: The British Lung Foundation (grant P07-2), LAM Action and the Jarron Family.
Conflict of interest: Disclosures can be found alongside the online version of this article at www.erj.ersjournals.com
- Received September 25, 2013.
- Accepted November 1, 2013.
- ©ERS 2014