Pneumologie 2023; 77(S 01): S13-S14
DOI: 10.1055/s-0043-1760895
Abstracts

New diagnostic approaches for pulmonary lymphangioleiomyomatosis

C Lederer
1   Zentrum für Interstitielle und Seltene Lungenerkrankungen, Pneumologie und Beatmungsmedizin, Thoraxklinik Heidelberg, Universitätsklinikum Heidelberg Und; Translationales Zentrum für Lungenforschung Heidelberg (Tlrc), Mitglied des Deutschen Zentrums für Lungenforschung (Dzl), Heidelberg, Deutschland
,
M Kreuter
2   Zentrum für Interstitielle und Seltene Lungenerkrankungen, Pneumologie und Beatmungsmedizin, Thoraxklinik; Universitätsklinikum Heidelberg und Translationales Zentrum für Lungenforschung Heidelberg (Tlrc); Mitglied des Deutschen Zentrums für Lungenforschung (Dzl)
,
C Heußel
3   Diagnostische und Interventionelle Radiologie Mit Nuklearmedizin, Thoraxklinik Heidelberg, Universitätsklinikum Heidelberg Und; Translationales Zentrum für Lungenforschung Heidelberg (Tlrc), Mitglied des Deutschen Zentrums für Lungenforschung (Dzl), Heidelberg, Deutschland
,
F Herth
4   University Hospital Heidelberg; Institute of Internal Medicin III – Pneumology; Thoraxklinik
,
V Müller
5   Semmelweis University Áok; Clinic of Pulmonology
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Background Lymphangioleiomyomatosis (LAM) is a rare multisystem disease primarily affecting females that is characterized by the formation of lung cysts. Clinical research in this disease area is sparse due to small patient numbers. Patient databases are therefore essential to facilitate research and improve patient care.

Aims We performed a combined retrospective and prospective study to establish a multicenter database of LAM patients. The database includes clinical, lung functional, laboratory, radiological, histological and treatment information. This data enables the evaluation of new diagnostic approaches to stratify disease severity and progression.

Methods We created a database of 80 female patients from two large ILD expert centers in Europe who received a LAM diagnosis based on ERS/ATS diagnostic criteria. The mean patient age at diagnosis is 43. We then applied a novel method to quantify cyst volume on high-resolution computed tomography (HRCT). Cyst volume on HRCT was calculated as the percentage of total lung volume using the software Yet Another CT-Analyzer (YACTA). The cyst volume was then correlated with pulmonary function tests (PFT)-parameters.

Results Mean cyst volume (n=55) was 10.5±10.8%, range: 0-44%. We found a significant negative correlation (r=-0.44, R2=0.19, p<0.001) between cyst volume index (CVI) and forced expiratory volume in the first second (FEV1). We also found a negative correlation (r=-0.44, R2=0.20, p<0.001) between CVI and diffusing capacity for carbon monoxide (DLCO) ([Abb. 1]).

Conclusion We created a comprehensive, multicenter database which to date includes 80 LAM patients. Quantification of CVI on HRCT using the software YACTA correlates with PFT parameters. Therefore, the CVI could be a useful index for risk stratification and prognosis estimation.

Zoom Image
Fig. 1 There was a significant negative correlation between CVI and FEV1 (a) as well as between CVI and DLCO (b), two well-established parameters for lung functional impairment in LAM patients. Cyst volume quantification was determined using YACTA-software on HRCT of LAM patients. CVI vs. FEV1: r= -0.44, R2=0.19, p<0.001, n=55. CVI vs. DLCO: r= -0.44, R2=0.20, p<0.001, n=55.CVI=cyst volume index in % of total lung volume; YACTA=Yet Another CT-Analyzer; HRCT=high-resolution computed tomography.


Publication History

Article published online:
09 March 2023

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