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. 2022 Sep 24;22(1):362.
doi: 10.1186/s12890-022-02123-7.

Computer grading of lung disease severity in patients with lymphangioleiomyomatosis referred for transplantation

Angelo M Taveira-DaSilva  1 , Vissaagan Gopalakrishnan  1 , Jianhua Yao  2 , Marcus Y Chen  3 , Patricia Julien-Williams  1 , Amanda M Jones  1 , Gustavo Pacheco-Rodriguez  1 , Joel Moss  4
Affiliations

Computer grading of lung disease severity in patients with lymphangioleiomyomatosis referred for transplantation

Angelo M Taveira-DaSilva et al. BMC Pulm Med. .

Abstract

Objectives: Lymphangioleiomyomatosis (LAM) patients with severe lung disease may be considered for lung transplantation. Clinical, physiologic, and quality of life data are usually employed for referral. The aim of this study was to determine whether computed tomographic measurement of lung volume occupied by cysts (cyst score) complemented clinical and physiologic data in supporting referral for transplantation.

Methods: Forty-one patients were studied. Pre-referral clinical data, pulmonary function tests, exercise testing, and high-resolution computed tomography (HRCT) scans were obtained. From HRCT, a computer-aided diagnostic program was employed to calculate cyst scores. These data were compared to those of 41 age-matched LAM patients not referred for lung transplantation.

Results: Cyst score, and % predicted FEV1 and DLCO were respectively, 48.1 ± 9.4%, 36.5 ± 9.1%, and 35.0 ± 10.7%. For the control group, cyst score, FEV1, and DLCO were respectively, 14.8 ± 8.3%, 77.2 ± 20.3%, and 66.7 ± 19.3%. Cyst score values showed a normal distribution. However, the frequency distribution of FEV1 was skewed to the right while the distribution of DLCO was bimodal. Correlations between cyst score and FEV1 and DLCO for the study group were respectively, r = - 0.319 and r = - 0.421.

Conclusions: LAM patients referred for lung transplantation had nearly 50% of lungs occupied by cysts. Correlations between cyst score and FEV1 or DLCO were weak; as shown previously, DLCO was better related to cyst number while FEV1 had a better association with cyst size. Given its normal distribution, cyst score measurements may assist in evaluation of pre-transplant severity of lung disease before referral for transplantation.

Keywords: Cystic lung disease; High resolution computed tomography (HRCT); Lung transplant; Lymphangioleiomyomatosis (LAM).

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Conflict of interest statement

None of the authors has any financial conflicts of interest.

Figures

Fig. 1
Fig. 1
Box-and whiskers plot showing percent cyst score, and corresponding percent predicted FEV1 and DLCO from 41 LAM patients who were referred for or underwent lung transplantation (white bars), and a group of 41 patients matched by age, who were not referred for transplantation evaluation (hatched bars). The top line represents the 75% quartile and the lower line represents the 25% quartile. The line across the boxes represents the second quartile (median). The upper and lower whiskers indicate the maximum and minimum values. The patients who were referred for or underwent lung transplantation have significantly higher cyst score and lower FEV1 and DLCO, *p < 0.001
Fig. 2
Fig. 2
Panels A–C. Frequency distribution of cyst scores (A), FEV1 (B), and DLCO (C), in 41 LAM patients with severe LAM. The frequency distribution of cyst scores is normal. Note that the frequency distribution of the FEV1 is skewed to the left and the frequency distribution of the DLCO, is bimodal
Fig. 3
Fig. 3
Panels A, B. Relationship between cyst score and FEV1 (A), and DLCO (B), in 41 LAM patients with severe lung disease referred for lung transplantation
Fig. 4
Fig. 4
A ROC curve of the percent cyst score from patients that could be defined to be referred for lung transplantation based on the data from both the study group and an age-based control group with less severe disease. Transplanted group, red line; control group, blue line
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