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Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.

ABSTRACT: Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.

SUBMITTER: Sekimoto Y

PROVIDER: S-EPMC8010110 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature

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Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.

Sekimoto Yasuhito Y Suzuki Kazuhiro K Okura Makiko M Hayashi Takuo T Ebana Hiroki H Kumasaka Toshio T Mitani Keiko K Nishino Koichi K Okamoto Shouichi S Kobayashi Etsuko E Takahashi Kazuhisa K Seyama Kuniaki K

Scientific reports 20210330 1

Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM ...[more]

PMID: 33785773

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Project description:ObjectivesWe aimed to study sirolimus-related lung parenchymal changes by quantitative analysis of computed tomography (CT) of the lungs in patients with lymphangioleiomyomatosis (LAM).MethodsWe studied 20 participants from the Multicenter Lymphangioleiomyomatosis Sirolimus Trial for Safety study, who had undergone both thin-section CT scans and pulmonary function tests at baseline, 12, and 24 months. Quantitative CT parameters such as CT-derived total lung capacity, percentage of low attenuation area (LAA%), lung density histogram, fractal property of low attenuation area, and airway dimensions were analyzed, and correlations were conducted between the longitudinal change in each quantitative CT measurement and changes in pulmonary function were examined. Among 20 participants, pre-trial (n = 8) and post-trial (n = 16) CT data were also analyzed to deduce pathophysiologic implications of the serial changes in CT parameters during trial periods.ResultsFEV1 significantly increased from baseline to 24 months (slope 3.71 ± 1.50 ml/month) whereas FVC didn't during sirolimus therapy. Strikingly, LAA%, and skewness and kurtosis of density histogram significantly increased from baseline to 24 months, while mean and mode CT values significantly decreased from baseline to 24 months. Statistically significant positive correlations were found between ΔFEV1 and Δskewness (r = 0.465, p = 0.045). Taking the changes in lung density during pre-trial period into consideration, sirolimus decreases the area of -800 to -750 Housefield unit (HU) density and inhibits the decrease of -950 to -800 HU area during treatment, then producing the increased LAA% during the trial and post-trial periods. Given few sirolimus-related changes in airway dimensions, possible changes in lung mechanics may have contributed to increased FEV1.ConclusionOur study suggests that the lung density histogram parameters, kurtosis, and skewness, may be useful as indicators of the efficacy of sirolimus.

Project description:ObjectiveChest computed tomography (CT) imaging is being increasingly used for potential lung donor assessment. However, the efficacy of CT imaging in this setting remains unknown. We hypothesize that chest CT imaging independently affects the decision-making process in donor lung utilization.MethodsWe conducted a retrospective cohort study of all adult donation after brain death donors managed through our local organ procurement organization from June 2011 to November 2016. An experienced thoracic radiologist independently reviewed donor chest CT and chest x-ray images in a blinded, standardized manner to determine the presence of structural lung disease (eg, emphysema, interstitial lung disease [ILD]) and acute abnormalities (eg, traumatic lung injury [TLI]). Distinct models of lung utilization were fit to groups with initial partial pressure of oxygen (iPaO2) ≤300 mm Hg (suboptimal) and iPaO2 >300 mm Hg (optimal).ResultsThe organ procurement organization managed 753 donors during the study period, with a lung utilization rate ([lung donors/all organ donors] × 100) of 36.5% (275 of 753). Four hundred forty-five (59.1%) donors received chest CT imaging, revealing emphysema (13.7%), ILD (2.5%), and TLI (7.2%). In univariate analysis, findings of TLI (odds ratio [OR], 2.23; 95% confidence interval [CI], 1.08-4.61) were positively associated with lung utilization, whereas findings of emphysema (OR, 0.18; CI, 0.08-0.40) were negatively associated with utilization. In multivariate analysis, CT findings of emphysema (OR, 0.21; CI 0.08-0.54) remained negatively associated with utilization. No potential donors with CT findings of ILD became lung donors. After controlling for chest x-ray findings, chest CT imaging findings of structural lung disease remained negatively associated with utilization (P = .0001). Lung utilization rate in the suboptimal and optimal iPaO2 populations was 35.1% and 41.4%, respectively, and CT findings of emphysema had a significant association with nonutilization in both groups.ConclusionsIn the evaluation of potential lung donors, chest CT imaging findings of structural lung disease, such as emphysema and ILD, have a significant negative association with lung utilization. Our findings suggest that chest CT imaging might be an important adjunct to conventional lung donor assessment criteria.

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Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.

Publications

Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.

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