Diffuse Lung Disease: Diffuse Lung Disease |

Successful Delivery in a Woman With LAM and Respiratory Failure FREE TO VIEW

Yating Peng, MD; Ruoyun Ouyang, PhD; Ping Chen, PhD; Dandan Zong, PhD; Li Zhou, MD
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Respiratory Disease Research Institute, Changsha, China

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A205. doi:10.1016/j.chest.2016.02.212
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SESSION TITLE: Diffuse Lung Disease

SESSION TYPE: Case Report Slide

PRESENTED ON: Saturday, April 16, 2016 at 05:15 PM - 06:15 PM

INTRODUCTION: LAM is a rare disease in women of childbearing age and is characterized by the proliferation of abnormal smooth muscle cells in the lungs. We report here a very rare case of a pregnant LAM woman who successfully gave cesarean birth to a baby despite severe respiratory failure.

CASE PRESENTATION: A 33-year-old woman developed dyspnea, pneumothorax and hemosputum in the 16th week of her third pregnancy. At the 31th week of pregnancy, the pneumothorax relapsed for the fifth time and was accompanied with severe hypoxia and hypocapnia. At the 33th week pregnancy she received an epidural anesthetic and uneventfully gave birth to a healthy female baby by cesarean section. HRCT examination 6 days after delivery showed small thin-walled cystic lesions diffuse in both lungs (Fig1). Blood sample was taken three weeks after delivery and showed serum VEGF-D level was 6608pg/ml. She accepted sirolimus treatment (2mg/day) thereafter and gradually recoveed.

DISCUSSION: LAM have long been considered as contraindications to pregnancy because of a worse outcome[1]. In the present case, the patient had a strong desire to have her baby. We conclude intense care, sufficient preparations before operation, proficient anesthesia, skilled nursing is necessary for the successful delivery of LAM patients. For pregnant women with LAM, a definite diagnosis is difficult according to ERS Guidelines. Unavailable radiological or invasive procedures may delay the exact diagnose. The optimal cut-off point for definite LAM diagnosis was 850.7 pg/ml and it was correlated with disease severity, presentations of chylous effusions as well as treatment responses to sirolimus [2]. For now, sirolimus or everolimus, two mTOR inhibitors, appear to be the most important, and the efficacy of sirolimus in LAM treatment has been proved. However, some serious adverse events like peripheral oedema, pneumonia, cardiac failure and Pneumocystis jirovecii infection were happened when everolimus (2.5 mg/day escalated to 10 mg/day[3]. We hope future research may cover a convenient diagnosis procedure, an effective risk/prognosis assessment as well as optimal management strategies for pregnant LAM patients.

CONCLUSIONS: In summary, we encountered a rare case of a pregnant LAM patient with respiratory failure successfully gave birth to a child. Cesarean section and sirolimus treatment controlled her symptoms. Periphery serum VEGF-D level may provide diagnose and disease evaluation utility in the future. Targeting VEGF-D or progesterone receptors may be a promising treatment for the LAM.

Reference #1: Cohen MM, Freyer AM, Johnson SR. Pregnancy experiences among women with lymphangioleiomyomatosis. Respir Med 2009;103:766-772.

Reference #2: Young L, Lee HS, Inoue Y, et al. Serum VEGF-D a concentration as a biomarker of LAM severity and treatment response: a prospective analysis of the MILES trial. Lancet Respir Med 2013;1:445-452.

Reference #3: Goldberg HJ, Harari S, Cottin V, et al. Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study. Eur Respir J 2015;46:783-794

DISCLOSURE: The following authors have nothing to disclose: Yating Peng, Ruoyun Ouyang, Ping Chen, Dandan Zong, Li Zhou

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