Evaluation of the Extent of Pulmonary Cysts and Their Association with Functional Variables and Serum Markers in Lymphangioleiomyomatosis (LAM).

[lymphangioleiomyomatosis]

Although computed tomography (CT ) has been used previously to assess disease severity in lymphangioleiomyomatosis (LAM ), the associations between the extent of pulmonary cysts on CT and six -minute walk test (6 MWT ), matrix metalloproteinases (MMPs ) and vascular endothelial growth factor (VEGF-D ) are not well established . We performed a cross-sectional study to quantify the extent of pulmonary cysts in CT and to establish their correlations with pulmonary function tests (PFTs ), 6 MWT results , including a composite index (desaturation-distance ratio , DDR ), and levels of VEGF-D and MMPs in LAM .Twenty -three LAM patients underwent CT scanning to automatically quantify the extent of pulmonary cysts and performed PFTs and 6 MWT . Serum levels of MMP-2 , MMP-9 , and VEGF-D were also measured .The severity of pulmonary cystic involvement was mild (the extent of cysts was 6 .8 Â %) and correlated best with FEV 1 /FVC (r Â =Â -0 .84 ), residual volume (r Â =Â 0 .66 ), DLCO (r Â =Â -0 .82 ), the DDR index (r Â =Â 0 .77 ), and desaturation during the 6 MWT (r Â =Â -0 .81 ). There was a weak correlation with VEGF-D (r Â =Â 0 .45 ), but no association was found with MMP-2 and MMP-9 .The severity of pulmonary cystic involvement was mild in this sample of LAM patients and correlated best with airway obstruction , air trapping , reduced DLCO , the DDR index , and desaturation during the 6 MWT . Serum VEGF-D can not be completely defined as a valuable marker of disease severity and there may be a mechanism independent of MMPs to explain the formation of pulmonary cysts .

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Diseases presenting "weak correlation" symptom

lymphangioleiomyomatosis

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