Abstract | BACKGROUND: OBJECTIVE: DESIGN: Observational study. SETTING: The National Institutes of Health Clinical Center. PATIENTS: 19 patients with rapidly progressing LAM or chylous effusions. INTERVENTION: MEASUREMENTS: RESULTS: 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 (P<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. LIMITATIONS: This was an observational study. The resolution of effusions may have affected improvements in lung function. CONCLUSION: PRIMARY FUNDING SOURCE: Intramural Research Program, National Heart, Lung, and Blood Institute, National Institutes of Health.
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Authors | Angelo M Taveira-DaSilva, Olanda Hathaway, Mario Stylianou, Joel Moss |
Journal | Annals of internal medicine
(Ann Intern Med)
Vol. 154
Issue 12
Pg. 797-805, W-292-3
(Jun 21 2011)
ISSN: 1539-3704 [Electronic] United States |
PMID | 21690594
(Publication Type: Journal Article, Research Support, N.I.H., Intramural)
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Chemical References |
- Antibiotics, Antineoplastic
- Immunosuppressive Agents
- TOR Serine-Threonine Kinases
- Sirolimus
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Topics |
- Adult
- Angiomyolipoma
(diagnostic imaging, drug therapy, physiopathology)
- Antibiotics, Antineoplastic
(adverse effects, blood, therapeutic use)
- Cell Proliferation
(drug effects)
- Chyle
(metabolism)
- Female
- Humans
- Immunosuppressive Agents
(adverse effects, blood, therapeutic use)
- Lung
(diagnostic imaging, physiopathology)
- Lung Neoplasms
(diagnostic imaging, drug therapy, physiopathology)
- Lymphangioleiomyomatosis
(diagnostic imaging, drug therapy, physiopathology)
- Lymphangiomyoma
(diagnostic imaging, drug therapy, physiopathology)
- Middle Aged
- Muscle, Smooth
(pathology)
- Observation
- Pleural Effusion
(physiopathology)
- Respiratory Function Tests
- Sirolimus
(adverse effects, blood, therapeutic use)
- TOR Serine-Threonine Kinases
(antagonists & inhibitors)
- Tomography, X-Ray Computed
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