Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis

Ellender, Claire; Williams, Trevor; Gooi, Julian; Snell, Gregory I; Whitford, Helen

doi:10.1002/rcr2.105

Cited by 6 publications

(8 citation statements)

References 8 publications

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“…Consistent with the speculation mentioned above, the prospective trials included in our study have reported the efficacy of sirolimus in the improvement or stabilization of lung function, amelioration of QOL scores, and reduction in AML volume [9, 24, 33-35, 37, 38, 40]. Furthermore, 1 observational study [46] and 7 case reports [47][48][49][50][51][52][53] also addressed the efficacy of sirolimus in the management of chylous effusions. In the present study, quantitative analyses of lung function in patients treated with sirolimus revealed that the changes in lung function and 6MWD values were not significant.…”

Section: Discussionsupporting

confidence: 74%

The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis

et al. 2020

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Background: Lymphangioleiomyomatosis (LAM) is a rare, low-grade multisystem neoplastic disease. Most LAM patients are at a high risk of losing lung function at an accelerated rate and developing progressive dyspnea. Recently, several studies have reported their experience with pharmacological treatments for LAM. Therefore, we conducted a systematic review and meta-analysis to assess the efficacy and safety of these therapies. Methods: PubMed (Medline), EMBASE, Cochrane Library, Web of Science and EBSCO Host were searched (until March 31, 2019) for eligible prospective studies regarding LAM patients treated with pharmacological treatments. Random effect models were used for quantitative analysis. Results: Fourteen prospective studies regarding five pharmacological treatments (including sirolimus, everolimus, doxycycline, triptorelin, and a combination therapy of sirolimus and hydroxychloroquine) were enrolled in our systematic review, and ten of them were used for the meta-analysis. Seven prospective studies reported that sirolimus was effective at improving or stabilizing lung function and alleviating renal angiomyolipoma (AML) in LAM patients. Subsequent quantitative analyses showed that during sirolimus treatment, the pooled values of lung function and 6-min walk distance (6MWD) were not significantly changed (P > 0.05), with the pooled response rate of AML being 0.62 (95% confidence intervals [CIs]: 0.43 to 0.82, I 2 = 65%). Regarding everolimus, three prospective studies reported similar effects to those of sirolimus with regard to preserving lung function and reducing AMLs. The meta-analysis showed that the changes in lung function during everolimus treatment were not statistically significant (P > 0.05), while the pooled response rate of AML was 0.78 (95% CI: 0.68 to 0.88, I 2 = 8%). Neither the qualitative nor the quantitative results confirmed the benefits of doxycycline or triptorelin treatment, and the effects of the combination therapy were unclear in LAM patients. Most of the adverse events during pharmacological treatments were low or moderate grade and tolerable. Conclusions: Overall, sirolimus and everolimus were recommended for the treatment of LAM because they could stabilize lung function and alleviate renal AML. Doxycycline and triptorelin were not recommended for the treatment of LAM because no beneficial outcomes were consistently observed. The efficacy and safety of combination therapy remain to be further explored.

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Section: Discussionsupporting

confidence: 74%

The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis

et al. 2020

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“…Sirolimus was recently reported to reduce refractory chylothorax and chylous ascites in patients with LAM ( 3 , 5 - 7 ). However, in our case, chylous effusion developed during sirolimus therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Since sirolimus inhibits the proliferation of LAM cells ( 9 ), it is also expected to reduce chylous effusion. Indeed, some reports have shown that chylous effusion was ameliorated by sirolimus ( 3 , 5 - 7 ). In the present case, the patient's lung function was improved a year after the start of sirolimus administration compared with that before the treatment, and multiple thin-walled cysts in both lung fields had not worsened on CT.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, chylothorax and chylous ascites occur in approximately 12% and 7% of LAM patients, respectively ( 2 ). Such chylous effusion is often refractory to medical and surgical management ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

“…Recently, the mTOR inhibitor sirolimus, which has been shown to stabilize the lung function in LAM patients ( 4 ), was reported to be effective in decreasing chylous effusion ( 3 , 5 - 7 ). However, there are a few LAM patients whose chylous effusion is refractory to sirolimus therapy.…”

Section: Introductionmentioning

confidence: 99%

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Additional Octreotide Therapy to Sirolimus Achieved a Decrease in Sirolimus-refractory Chylous Effusion Complicated with Lymphangioleiomyomatosis

et al. 2017

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Recently, sirolimus, an inhibitor of mammalian target of rapamycin, was reported to decrease chylous effusion in patients with lymphangioleimyomatosis (LAM). We herein report a case of a 34-year-old woman with LAM who developed refractory chylothorax and chylous ascites during sirolimus therapy. In this case, to reduce chylous effusion, we administered octreotide, which is often used to control postoperative chylous effusion, in addition to the sirolimus therapy. This combination therapy reduced the chylothorax and chylous ascites. For patients with LAM, octreotide therapy in addition to sirolimus may be effective for treating sirolimus-refractory chylous effusion.

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Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management

McCormack¹,

Gupta²,

Finlay³

et al. 2016

Am J Respir Crit Care Med

268

289

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Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis

Cited by 6 publications

References 8 publications

The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis

The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis

Additional Octreotide Therapy to Sirolimus Achieved a Decrease in Sirolimus-refractory Chylous Effusion Complicated with Lymphangioleiomyomatosis

Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management

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