Pregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature

Khaddour, Karam; Shayuk, Maryna; Ludhwani, Dipesh; Gowda, Satish; Ward, Wendy L.

doi:10.1016/j.rmcr.2018.11.010

Cited by 11 publications

(14 citation statements)

References 40 publications

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“…Exacerbated dyspnea, pneumothorax, kidney tumors, or retroperitoneal masses during pregnancy may be the first symptoms of patients with LAM [ 10 – 12 ] [ 13 ]. These patients have been described as previously healthy or with a history of asthma or kidney tumors or retroperitoneal mass that had not been noticed before.…”

Section: Discussionmentioning

confidence: 99%

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Shen

Gao

et al. 2021

Orphanet J Rare Dis

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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.

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Section: Discussionmentioning

confidence: 99%

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Shen

Gao

et al. 2021

Orphanet J Rare Dis

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“…Elevated serum VEGF-D in 70% of patients with LAM is a clinically useful diagnostic and prognostic biomarker. Furthermore, sirolimus, a therapeutic drug that inhibits VEGF-D and anti-lymphocyte proliferation, is highly effective in stabilizing lung function and minimizing complications in cases of LAM 9,10,19,20 .…”

Section: Developmentmentioning

confidence: 99%

Lymphangioleiomyomatosis: A Literature Overview

Maciel¹,

Azzi²,

Joia³

et al. 2023

JHS

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Lymphangioleiomyomatosis is a rare multisystem disease associated with genetic mutations. The disease usually occurs in women of reproductive age and is characterized by infiltration of immature smooth muscle cells into the lungs, airways, and axial lymphatic systems of the thorax and abdomen. The disease often destroys the lung parenchyma and produces air cysts. Cellular infiltration of lymphangioleiomyomatosis into the lymphatic axis can affect hilar lymph nodes, mediastinal nodes, and extrathoracic lymph nodes. The disease can cause lymphatic dilation in the lungs and thoracic ducts, causing chylous effusion in the pleural or abdominal cavities. Invasion of cells in the walls of pulmonary veins can lead to venous obstruction and pulmonary venous hypertension with hemoptysis. Most patients have cough, dyspnea, pneumothorax, hemoptysis, and abnormal lung function. Definitive diagnosis is usually based on histopathology and immunohistochemistry. We expect that, in the near future, quantitative blood tests for the growth factors VEGF-C and VEGF-D will become commonplace. Then the disease can be diagnosed more easily and in greater numbers.

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“…For example, as pregnancy is characterized by a pro-thrombotic state, hereditary thrombophilia is often unmasked by venous thrombosis in pregnancy [ 146 ]. Pregnancy can also trigger or exacerbate symptoms of undiagnosed lymphangioleiomyomatosis, a rare cystic lung disease that affects premenopausal women [ 147 , 148 ]. Thus, pregnancy can be viewed as a stress test to identify risks of future disease and is therefore a critical period to implement primary preventative care and enhanced follow-up measures for women at high risk for certain diseases, such as cardiovascular disease [ 149 ].…”

Section: Potential Mechanisms Linking Acquisition Of Ti After Exposur...mentioning

confidence: 99%

Cross-Generational Impact of Innate Immune Memory Following Pregnancy Complications

Lodge-Tulloch

Toews

Atallah

et al. 2022

Cells

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Pregnancy complications can have long-term negative effects on the health of the affected mothers and their children. In this review, we highlight the underlying inflammatory etiologies of common pregnancy complications and discuss how aberrant inflammation may lead to the acquisition of innate immune memory. The latter can be described as a functional epigenetic reprogramming of innate immune cells following an initial exposure to an inflammatory stimulus, ultimately resulting in an altered response following re-exposure to a similar inflammatory stimulus. We propose that aberrant maternal inflammation associated with complications of pregnancy increases the cross-generational risk of developing noncommunicable diseases (i.e., pregnancy complications, cardiovascular disease, and metabolic disease) through a process mediated by innate immune memory. Elucidating a role for innate immune memory in the cross-generational health consequences of pregnancy complications may lead to the development of novel strategies aimed at reducing the long-term risk of disease.

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Pregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature

Cited by 11 publications

References 40 publications

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis: A Literature Overview

Cross-Generational Impact of Innate Immune Memory Following Pregnancy Complications

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