Successful Octreotide Treatment of Chylous Pleural Effusion and Lymphedema in the Yellow Nail Syndrome

Makrilakis, Konstantinos; Pavlatos, Spyridon; Giannikopoulos, George; Toubanakis, Christos; Katsilambros, Nikolaos

doi:10.7326/0003-4819-141-3-200408030-00028

Cited by 27 publications

(17 citation statements)

References 4 publications

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“…As our study demonstrates, chylous effusions in LAM are difficult to treat and repeated thoracentesis may lead to severe weight loss. Other therapies, such as pleuroperitoneal shunts and octreotide, have been tried, but there is little experience with these therapeutic methods in patients with LAM (43–45). Although lymphangioleiomyomas are usually asymptomatic, compression of the bladder, bowel, pelvic veins, nerves, and other organs may cause pain, obstipation, frequent urination, and peripheral edema.…”

Section: Discussionmentioning

confidence: 99%

Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus

Taveira-DaSilva

Hathaway²,

Stylianou³

et al. 2011

Ann Intern Med

177

125

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Background 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. Objective 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. Design Observational study. Setting The National Institutes of Health Clinical Center. Patients 19 patients with rapidly progressing LAM or chylous effusions. Intervention Treatment with sirolimus. Measurements Lung function and the size of chylous effusions and lymphangioleiomyomas before and during sirolimus therapy. 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 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. Primary Funding Source Intramural Research Program, National Heart, Lung, and Blood Institute, National Institutes of Health.

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Section: Discussionmentioning

confidence: 99%

Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus

Taveira-DaSilva

Hathaway²,

Stylianou³

et al. 2011

Ann Intern Med

177

125

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“…MCT diet has been successfully used to treat a variety of disorders, including primary lymphangiectasia, traumatic chylous duct injury and recurrent chylous effusions in LAM [8]. Octreotide, a somatostatin analogue, has been used with variable success in a variety of chylous disorders [9]. Although the mechanism of action of octreotide is unclear, it is believed to prevent splanchnic vasoconstriction and inhibits absorption of triglycerides, thus reducing lymphatic flow.…”

Section: Successful Treatment Of Progressive Diffuse Pecomatosismentioning

confidence: 99%

Successful treatment of progressive diffuse PEComatosis

Lawson¹,

Maher

Hansell

et al. 2012

Eur Respir J

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“…We showed him two individual case reports describing benefit, not only in controlling pleural effusions, but also in reducing lymphedema (2,3). We showed him two individual case reports describing benefit, not only in controlling pleural effusions, but also in reducing lymphedema (2,3).…”

Section: Controlling the Pleural Effusionmentioning

confidence: 78%

“You Have a Syndrome”—Words You Don’t Want to Hear from a Doctor. Battling Yellow Nail Syndrome

Bauer¹,

Bauer²

2014

Annals ATS

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Successful Octreotide Treatment of Chylous Pleural Effusion and Lymphedema in the Yellow Nail Syndrome

Cited by 27 publications

References 4 publications

Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus

Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus

Successful treatment of progressive diffuse PEComatosis

“You Have a Syndrome”—Words You Don’t Want to Hear from a Doctor. Battling Yellow Nail Syndrome

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