{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T19:10:31Z","timestamp":1774120231773,"version":"3.50.1"},"reference-count":30,"publisher":"European Respiratory Society (ERS)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2013,11,1]]},"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:p>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.<\/jats:p><jats:p>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.<\/jats:p><jats:p>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\u00b1<jats:sc>sd<\/jats:sc>decline in FEV<jats:sub>1<\/jats:sub>, the primary endpoint, did not differ between the groups being -90\u00b1154 mL\u00b7year<jats:sup>\u22121<\/jats:sup>in the placebo group and -123\u00b1246 mL\u00b7year<jats:sup>\u22121<\/jats:sup>in the doxycycline group (difference -32.5, 95% CI -213\u2013148; 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).<\/jats:p><jats:p>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.<\/jats:p>","DOI":"10.1183\/09031936.00167413","type":"journal-article","created":{"date-parts":[[2013,12,6]],"date-time":"2013-12-06T02:08:36Z","timestamp":1386295716000},"page":"1114-1123","source":"Crossref","is-referenced-by-count":55,"title":["A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis"],"prefix":"10.1183","volume":"43","author":[{"given":"William Y.C.","family":"Chang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer L.","family":"Cane","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maruti","family":"Kumaran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"Lewis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne E.","family":"Tattersfield","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon R.","family":"Johnson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"81","published-online":{"date-parts":[[2013,12,5]]},"reference":[{"key":"2024102022211847000_43.4.1114.1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.06.00113303"},{"key":"2024102022211847000_43.4.1114.2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.160.2.9901027"},{"key":"2024102022211847000_43.4.1114.3","doi-asserted-by":"publisher","DOI":"10.1378\/chest.126.6.1867"},{"key":"2024102022211847000_43.4.1114.4","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1100391"},{"key":"2024102022211847000_43.4.1114.5","doi-asserted-by":"publisher","DOI":"10.1172\/JCI58709"},{"key":"2024102022211847000_43.4.1114.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa063564"},{"key":"2024102022211847000_43.4.1114.7","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-11-0445"},{"key":"2024102022211847000_43.4.1114.8","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2006-0372TR"},{"key":"2024102022211847000_43.4.1114.9","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2008.189"},{"key":"2024102022211847000_43.4.1114.10","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00022.2006"},{"key":"2024102022211847000_43.4.1114.11","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2008.07.017"},{"key":"2024102022211847000_43.4.1114.12","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-13-34"},{"key":"2024102022211847000_43.4.1114.13","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(97)90061-7"},{"key":"2024102022211847000_43.4.1114.14","doi-asserted-by":"crossref","first-page":"267","DOI":"10.5858\/2000-124-0267-RFAOMM","article-title":"Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis","volume":"124","author":"Matsui","year":"2000","journal-title":"Arch Pathol Lab Med"},{"key":"2024102022211847000_43.4.1114.15","doi-asserted-by":"publisher","DOI":"10.1177\/08959374980120010501"},{"key":"2024102022211847000_43.4.1114.16","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64929-2"},{"key":"2024102022211847000_43.4.1114.17","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc053410"},{"key":"2024102022211847000_43.4.1114.18","doi-asserted-by":"publisher","DOI":"10.1590\/S1806-37132011000400003"},{"key":"2024102022211847000_43.4.1114.19","doi-asserted-by":"publisher","DOI":"10.1590\/S1806-37132013000100002"},{"key":"2024102022211847000_43.4.1114.20","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00076209"},{"key":"2024102022211847000_43.4.1114.21","doi-asserted-by":"publisher","DOI":"10.1016\/S0954-6111(05)80210-0"},{"key":"2024102022211847000_43.4.1114.22","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2008.01.006"},{"key":"2024102022211847000_43.4.1114.23","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-0573"},{"key":"2024102022211847000_43.4.1114.24","unstructured":"DCTD, NCI, NIH, DHHS. 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