Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Abstract

Background Lymphangioleiomyomatosis (LAM) is a rare disease that almost exclusively affects women of reproductive age. Patients are warned of the increased risks if they become pregnant. However, information on pregnancy in patients after the diagnosis of LAM is limited. Methods Patients were collected from the LAM registry study at Peking Union Medical College Hospital, Beijing, China. Patients with a history of pregnancy after the diagnosis of LAM were included. Medical records were reviewed, and baseline information and data during and after pregnancy were collected in May 2018. Results Thirty patients with a total of 34 pregnancies after the diagnosis of LAM were included. Livebirth, spontaneous abortion and induced abortion occurred in 10, 6 and 18 pregnancies, respectively. Sirolimus treatment was common (17/34). A total of 6/10, 5/6, and 6/18 patients with livebirths, spontaneous abortions, and induced abortions respectively, had a history of sirolimus treatment. Ten pregnancies (29.4%) had LAM-associated complications during pregnancy, including the exacerbation of dyspnea in 7 patients, pneumothorax in 3 patients (2 resulting in induced abortion and 1 successful parturition), and spontaneous bleeding of renal angiomyolipomas in 2 patients (both having successful parturition). No chylothorax was found during pregnancy. There were six pregnancies in six patients (17.6%) who had a history of livebirth after sirolimus treatment for LAM (all having successful parturition and healthy infants); two of these patients reported exacerbated dyspnea after parturition compared with before pregnancy. Conclusions Patients with LAM, especially those taking sirolimus before pregnancy, were at a higher risk of spontaneous abortion. Complications such as pneumothorax, bleeding of renal angiomyolipoma, and exacerbated dyspnea during pregnancy were common. In patients without spontaneous abortion, sirolimus discontinuation before or during pregnancy did not lead to increased adverse neonatal outcomes.

Pulmonary lymphangioleiomyomatosis (LAM): A literature overview and case report

“…Approximately 50% of patients have a pneumothorax at the time of presentation, and unilateral or bilateral pleural effusions are present in 10%-20% of cases. Approximately 10%-25% of patients have normal radiographs at the time of examination despite the presence of multiple pulmonary cysts [21] , [22] , [23] .…”

Minimally invasive thoracoscopic surgery as a diagnostic and therapeutic approach in bilateral pneumothorax in pregnancy caused by lymphangioleiomyomatosis: A case report

“…Complications connected to LAM, including pneumothorax and chylothorax, may occur in pregnancy, and the possible connection between pregnancy and the progression of LAM is cause for serious concern. Also, it has been recorded that women with LAM have a poorer outcome of pregnancy, including a greater number of premature births and miscarriages [6,15,16].…”

“…Komplikacije povezane sa limfangioleiomiomatozom, uključujući pneumotoraks i hilotoraks, mogu se javiti tokom trudnoće, a izražena je zabrinutost zbog moguće povezanosti trudnoće sa progresijom limfangioleiomiomatoze. Takođe je zabeleženo da žene sa LAM-om imaju lošiji ishod trudnoće, uključujući i više prevremenih porođaja i abortusa [6,15,16].…”

“…Lečenje pneumotoraksa u trudnoći zavisi od stepena i faze progresije. Obično se koristi torakalna drenaža, sa ili bez aktivne aspiracije [16]. U slučaju naše pacijentkinje, dijagnoza limfangioleiomiomatoze nije bila poznata prilikom donošenja odluke o inicijalnom zbrinjavanju pneumotoraksa, pa smo koristili preporuke zvaničnih smernica za lečenje pneumotoraksa u trudnoći [23].…”

Maternal sirolimus therapy and fetal growth restriction

“…The effect on birth weight has not been mentioned in this series. Shen et al has described three pregnancies while on sirolimus for lymphangioleiomyomatosis [ 10 ]. However, treatment was discontinued in all cases before the first trimester.…”