case_images: PMC10895031_01
case_images: Timeline of case 1. CT, Computed tomography; MRI, Magnetic resonance imaging, HRCT, High-resolution computed tomography; FEV1, Forced expiratory volume in 1 s; FVC, Forced vital capacity; DLCO, Diffusion capacity for the lungs for carbon monoxide; LAM, Lymphangioleiomyomatosis; ICS, Inhaled corticosteroids; LABA, Long-acting beta-agonist; VEGFR, Vascular endothelial growth factor receptor.
case_images: fmed-11-1328471-g001.jpg
case_images: PMC10895031_01_fmed-11-1328471-g001.jpg
case_images: fig1
case_images: The course of the disease is outlined in Figure 1. She was a smoker, and pulmonary function tests (PFTs) were obstructive (Figure 1). At the time, PFTs were still obstructive, but with an increase in forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) (Figure 1). LAM. Later, the LAM diagnosis was supported by an elevated VEGF-D (Figure 1). Pulmonary function tests showed severe obstructive reduction and a low diffusion capacity for carbon monoxide (DLCO) (Figure 1).
case_images: Magnetic resonance imaging from case 1 from 2020 at diagnosis of leiomyoma (A) and 2023 at diagnosis of lymphangioleiomyomatosis (B). The tumor is indicated by the yellow arrow and enlarged in the yellow box.
case_images: fmed-11-1328471-g002.jpg
case_images: PMC10895031_01_fmed-11-1328471-g002.jpg
case_images: fig2
case_images: In 2020, CT was supplemented with magnetic resonance imaging (MRI), which raised suspicion of a benign leiomyoma infiltrating both the aorta and the inferior vena cava (Figure 2). The patient was regularly followed with an MRI, showing regression of the tumor (Figure 2).
case_images: High-resolution computed tomography from case 1 (A) and case 2 (B).
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case_images: PMC10895031_01_fmed-11-1328471-g003.jpg
case_images: fig3
case_images: The HRCT showed similar findings as before but this time interpreted as cysts and not emphysematous bullae (Figure 3A). An HRCT was performed showing multiple cysts compatible with LAM (Figure 3B).
case_images: Timeline of case 2. HRCT, High-resolution computed tomography; FEV1, Forced expiratory volume in 1 s; FVC, Forced vital capacity; DLCO, Diffusion capacity for the lungs for carbon monoxide; LAM, Lymphangioleiomyomatosis; BAL, Bronchoalveolar lavage; SABA, Short-acting beta agonist; ICS, Inhaled corticosteroids; LABA, Long-acting beta-agonist. aPulmonary function tests performed in 2014. bResult from VEGF-D analysis was available 4 months after BAL was performed.
case_images: fmed-11-1328471-g004.jpg
case_images: PMC10895031_01_fmed-11-1328471-g004.jpg
case_images: fig4
case_images: The course of the disease is outlined in Figure 4. There, PFTs showed severe obstruction and DLCO reduction (Figure 4). As indicated in Figure 4, her lung function has been stable since the initiation of the treatment.