Dataset Information

Ultra-Small Lung Cysts Impair Diffusion Without Obstructing Air Flow in Lymphangioleiomyomatosis.

ABSTRACT:

Background

Lymphangioleiomyomatosis (LAM) is a rare lung disease found primarily in women of childbearing age, characterized by the formation of air-filled cysts, which may be associated with reductions in lung function. An experimental, regional ultra-high resolution CT scan identified an additional volume of cysts relative to standard chest CT imaging, which consisted primarily of ultra-small cysts.

Research question

What is the impact of these ultra-small cysts on the pulmonary function of patients with LAM?

Study design and methods

A group of 103 patients with LAM received pulmonary function tests and a CT examination in the same visit. Cyst score, the percentage lung volume occupied by cysts, was measured by using commercial software approved by the US Food and Drug Administration. The association between cyst scores and pulmonary function tests of diffusing capacity of the lungs for carbon monoxide (Dlco) (% predicted), FEV₁ (% predicted), and FEV₁/FVC (% predicted) was assessed with statistical analysis adjusted for demographic variables. The distributions of average cyst size and ultra-small cyst fraction among the patients were evaluated.

Results

The additional cyst volume identified by the experimental, higher resolution scan consisted of cysts of 2.2 ± 0.8 mm diameter on average and are thus labeled the "ultra-small cyst fraction." It accounted for 27.9 ± 19.0% of the total cyst volume among the patients. The resulting adjusted, whole-lung cyst scores better explained the variance of Dlco (P < .001 adjusted for multiple comparisons) but not FEV₁ and FEV₁/FVC (P = 1.00). The ultra-small cyst fraction contributed to the reduction in Dlco (P < .001) but not to FEV₁ and FEV₁/FVC (P = .760 and .575, respectively). The ultra-small cyst fraction and average cyst size were correlated with cyst burden, FEV₁, and FEV₁/FVC but less with Dlco.

Interpretation

The ultra-small cysts primarily contributed to the reduction in Dlco, with minimal effects on FEV₁ and FEV₁/FVC. Patients with lower cyst burden and better FEV₁ and FEV₁/FVC tended to have smaller average cyst size and higher ultra-small cyst fraction.

Clinical trial registration

ClinicalTrials.gov; No.: NCT00001465; URL: www.clinicaltrials.gov.

SUBMITTER: Matthew BP

PROVIDER: S-EPMC8295912 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Many groups developed the methods to quantitatively analyze low attenuation area (LAA) on chest CT in patients with cystic lung diseases. Especially in COPD, it was reported that the cumulative size distribution of LAA clusters follows a power law characterized by the exponent D, which reflect the fractal dimension of terminal airspace geometry. We hypoyhesized that the quantitative charateristics of LAA clusters including fractal property might indicate the different features of the progression of cysts in cystic lung diseases. The aim of this study was to apply the CT image-based method of characterizing the size distribution of LAA clusters for lymphangioleiomyomatosis (LAM) and Birt-Hogg-Dubé syndrome (BHDS) to disclose their features of the progression of pulmonary cysts. 40 patients with COPD, 52 patients with LAM, and 18 patients with BHDS who had undergone CT scans at our institute between January 2002 and August 2009 were included. Differences among these diseases in the quantitative characteristics of LAA clusters {i.e., extent, number, size, fractal property, and the relationship between these quantitatives} were assessed. The Chi-sqsuare test, unpaired t-test, and one-way analyses of variance with Tukey post-hoc tests were used to compare groups, spline model with an interaction terms were used to assess the relationship between extent and number, and exponential regression model was used to assess the relationship between extent and size. Statistically significant differences separated the three diseases in extent and number (P < 0.001). Number was significantly correlated with extent in COPD (P < 0.001), but was not so in LAM and BHDS when extent exceeded 11.5% and 20.8%, respectively. Size was significantly correlated with extent in COPD and LAM (P < 0.001), but was not so in BHDS. The percentage of CT images with fractal property was higher in COPD than that in LAM and BHDS (95.8%, 92.9% and 63.0%, respectively). In conclusion, our study has demonstrated for the first time the different characteristics of the size distribution of LAA clusters among COPD, LAM and BHDS, and indicated that this method is useful for exploration of the pathophysiology in cystic lung diseases.