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Primary endpoints were safety, pharmacokinetics and serum vascular endothelial growth factor-D (VEGF-D) levels; secondary endpoints were measures of lung function.\n                  <\/jats:p>\n                  <jats:p>\n                    Following 26\u2005weeks of everolimus treatment, forced vital capacity exhibited stability, while forced expiration volume in 1\u2005s improved from baseline, with mean changes (95% confidence interval) of 10\u2005mL (\u2212111\u2013132) and 114\u2005mL (11\u2013217), respectively; 6-min walk distance improved by 47\u2005m. Median VEGF-D and collagen IV levels decreased from baseline, from 1730\u2005pg\u00b7mL\n                    <jats:sup>\u22121<\/jats:sup>\n                    to 934.5\u2005pg\u00b7mL\n                    <jats:sup>\u22121<\/jats:sup>\n                    , and 103\u2005ng\u00b7mL\n                    <jats:sup>\u22121<\/jats:sup>\n                    to 80.5\u2005ng\u00b7mL\n                    <jats:sup>\u22121<\/jats:sup>\n                    , respectively. Adverse events were mostly grade 1\u22122; mouth ulceration, headache, nausea, stomatitis and fatigue were common. Serious adverse events suspected to be treatment related included peripheral oedema, pneumonia, cardiac failure and\n                    <jats:italic>Pneumocystis jirovecii<\/jats:italic>\n                    infection. Everolimus blood levels increased dose proportionally.\n                  <\/jats:p>\n                  <jats:p>In this study, everolimus improved some measures of lung function and exercise capacity and reduced serum VEGF-D and collagen IV. Side effects were generally consistent with known toxicities of mTOR inhibitors, although some were severe.<\/jats:p>","DOI":"10.1183\/09031936.00210714","type":"journal-article","created":{"date-parts":[[2015,7,3]],"date-time":"2015-07-03T12:27:09Z","timestamp":1435926429000},"page":"783-794","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":87,"title":["Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study"],"prefix":"10.1183","volume":"46","author":[{"given":"Hilary J.","family":"Goldberg","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sergio","family":"Harari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ivan 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