Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus
Identifieur interne : 004453 ( Ncbi/Checkpoint ); précédent : 004452; suivant : 004454Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus
Auteurs : Angelo M. Taveira-Dasilva ; Olanda Hathaway ; Mario Stylianou ; Joel MossSource :
- Annals of internal medicine [ 0003-4819 ] ; 2011.
Abstract
Lymphangioleiomyomatosis (LAM) is a disorder that affects women and is characterized by cystic lung destruction, chylous effusions, lymphangioleiomyomas, and angiomyolipomas. It is caused by proliferation of abnormal smooth muscle–like cells. Sirolimus is a mammalian target of rapamycin inhibitor that has been reported to decrease the size of neoplastic growths in animal models of tuberous sclerosis complex and to reduce the size of angiomyolipomas and stabilize lung function in humans.
To assess whether sirolimus therapy is associated with improvement in lung function and a decrease in the size of chylous effusions and lymphangioleiomyomas in patients with LAM.
Observational study.
The National Institutes of Health Clinical Center.
19 patients with rapidly progressing LAM or chylous effusions.
Treatment with sirolimus.
Lung function and the size of chylous effusions and lymphangioleiomyomas before and during sirolimus therapy.
Over a mean of 2.5 years before beginning sirolimus therapy, the mean (±SE) FEV1 decreased by 2.8% ± 0.8% predicted and diffusing capacity of the lung for carbon monoxide (DLCO) decreased by 4.8% ± 0.9% predicted per year. In contrast, over a mean of 2.6 years of sirolimus therapy, the mean (± SE) FEV1 increased by 1.8% ± 0.5% predicted and DLCO increased by 0.8% ± 0.5% predicted per year (
This was an observational study. The resolution of effusions may have affected improvements in lung function.
Sirolimus therapy is associated with improvement or stabilization of lung function and reduction in the size of chylous effusions and lymphangioleiomyomas in patients with LAM.
Intramural Research Program, National Heart, Lung, and Blood Institute, National Institutes of Health.
Url:
DOI: 10.1059/0003-4819-154-12-201106210-00007
PubMed: 21690594
PubMed Central: 3176735
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus</title><author><name sortKey="Taveira Dasilva, Angelo M" sort="Taveira Dasilva, Angelo M" uniqKey="Taveira Dasilva A" first="Angelo M." last="Taveira-Dasilva">Angelo M. Taveira-Dasilva</name></author><author><name sortKey="Hathaway, Olanda" sort="Hathaway, Olanda" uniqKey="Hathaway O" first="Olanda" last="Hathaway">Olanda Hathaway</name></author><author><name sortKey="Stylianou, Mario" sort="Stylianou, Mario" uniqKey="Stylianou M" first="Mario" last="Stylianou">Mario Stylianou</name></author><author><name sortKey="Moss, Joel" sort="Moss, Joel" uniqKey="Moss J" first="Joel" last="Moss">Joel Moss</name></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">21690594</idno><idno type="pmc">3176735</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3176735</idno><idno type="RBID">PMC:3176735</idno><idno type="doi">10.1059/0003-4819-154-12-201106210-00007</idno><date when="2011">2011</date><idno type="wicri:Area/Pmc/Corpus">002973</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002973</idno><idno type="wicri:Area/Pmc/Curation">002972</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">002972</idno><idno type="wicri:Area/Pmc/Checkpoint">003054</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">003054</idno><idno type="wicri:Area/Ncbi/Merge">004453</idno><idno type="wicri:Area/Ncbi/Curation">004453</idno><idno type="wicri:Area/Ncbi/Checkpoint">004453</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus</title><author><name sortKey="Taveira Dasilva, Angelo M" sort="Taveira Dasilva, Angelo M" uniqKey="Taveira Dasilva A" first="Angelo M." last="Taveira-Dasilva">Angelo M. Taveira-Dasilva</name></author><author><name sortKey="Hathaway, Olanda" sort="Hathaway, Olanda" uniqKey="Hathaway O" first="Olanda" last="Hathaway">Olanda Hathaway</name></author><author><name sortKey="Stylianou, Mario" sort="Stylianou, Mario" uniqKey="Stylianou M" first="Mario" last="Stylianou">Mario Stylianou</name></author><author><name sortKey="Moss, Joel" sort="Moss, Joel" uniqKey="Moss J" first="Joel" last="Moss">Joel Moss</name></author></analytic><series><title level="j">Annals of internal medicine</title><idno type="ISSN">0003-4819</idno><idno type="eISSN">1539-3704</idno><imprint><date when="2011">2011</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background</title><p id="P1">Lymphangioleiomyomatosis (LAM) is a disorder that affects women and is characterized by cystic lung destruction, chylous effusions, lymphangioleiomyomas, and angiomyolipomas. It is caused by proliferation of abnormal smooth muscle–like cells. Sirolimus is a mammalian target of rapamycin inhibitor that has been reported to decrease the size of neoplastic growths in animal models of tuberous sclerosis complex and to reduce the size of angiomyolipomas and stabilize lung function in humans.</p></sec><sec id="S2"><title>Objective</title><p id="P2">To assess whether sirolimus therapy is associated with improvement in lung function and a decrease in the size of chylous effusions and lymphangioleiomyomas in patients with LAM.</p></sec><sec id="S3"><title>Design</title><p id="P3">Observational study.</p></sec><sec id="S4"><title>Setting</title><p id="P4">The National Institutes of Health Clinical Center.</p></sec><sec id="S5"><title>Patients</title><p id="P5">19 patients with rapidly progressing LAM or chylous effusions.</p></sec><sec id="S6"><title>Intervention</title><p id="P6">Treatment with sirolimus.</p></sec><sec id="S7"><title>Measurements</title><p id="P7">Lung function and the size of chylous effusions and lymphangioleiomyomas before and during sirolimus therapy.</p></sec><sec id="S8"><title>Results</title><p id="P8">Over a mean of 2.5 years before beginning sirolimus therapy, the mean (±SE) FEV<sub>1</sub> decreased by 2.8% ± 0.8% predicted and diffusing capacity of the lung for carbon monoxide (D<sub>LCO</sub>) decreased by 4.8% ± 0.9% predicted per year. In contrast, over a mean of 2.6 years of sirolimus therapy, the mean (± SE) FEV<sub>1</sub> increased by 1.8% ± 0.5% predicted and D<sub>LCO</sub> increased by 0.8% ± 0.5% predicted per year (<italic>P</italic> < 0.001). After beginning sirolimus therapy, 12 patients with chylous effusions and 11 patients with lymphangioleiomyomas experienced almost complete resolution of these conditions. In 2 of the 12 patients, sirolimus therapy enabled discontinuation of pleural fluid drainage.</p></sec><sec id="S9"><title>Limitations</title><p id="P9">This was an observational study. The resolution of effusions may have affected improvements in lung function.</p></sec><sec id="S10"><title>Conclusion</title><p id="P10">Sirolimus therapy is associated with improvement or stabilization of lung function and reduction in the size of chylous effusions and lymphangioleiomyomas in patients with LAM.</p></sec><sec id="S11"><title>Primary Funding Source</title><p id="P11">Intramural Research Program, National Heart, Lung, and Blood Institute, National Institutes of Health.</p></sec></div></front></TEI><affiliations><list></list><tree><noCountry><name sortKey="Hathaway, Olanda" sort="Hathaway, Olanda" uniqKey="Hathaway O" first="Olanda" last="Hathaway">Olanda Hathaway</name><name sortKey="Moss, Joel" sort="Moss, Joel" uniqKey="Moss J" first="Joel" last="Moss">Joel Moss</name><name sortKey="Stylianou, Mario" sort="Stylianou, Mario" uniqKey="Stylianou M" first="Mario" last="Stylianou">Mario Stylianou</name><name sortKey="Taveira Dasilva, Angelo M" sort="Taveira Dasilva, Angelo M" uniqKey="Taveira Dasilva A" first="Angelo M." last="Taveira-Dasilva">Angelo M. Taveira-Dasilva</name></noCountry></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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