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

Abstract

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 6MWT. Serum VEGF-D cannot 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.

Oxygen desaturation during a 6-minute walk test as a predictor of maximal exercise-induced gas exchange abnormalities in sarcoidosis

“…Measurement of ∆SpO 2 -6MWT as performed in the present study has recently been added to the international guidelines for walking tests (12). Although this test has not been investigated extensively, it is reproducible (13) and correlates with symptom intensity (14), results of pulmonary function testing (15), changes on computed tomography (CT) scans (16), and prognosis (17)(18)(19) in interstitial lung diseases. However, the correlation between ∆SpO 2 -6MWT and peak AaDO 2 and, in particular, the ability of ∆SpO 2 -6MWT to predict peak AaDO 2 , are unknown.…”

Correlation of serum vascular endothelial growth factor-D concentration with clinical presentation and course of lymphangioleiomyomatosis

“…It was shown, that angiomyolipomas presence influenced serum VEGF-D concentration [13,23]. Positive correlation between the higher level of serum VEGF-D and a size of the angiomyolipoma was revealed [13,22]. In our study there was a trend that patients with sLAM and renal AML had higher values of VEGF-D, but the volume of lesions was not analysed.…”

“…Xu et al, in a study of 78 cases, did not find correlations between VEGF-D and PaO2, 6-MWT, FEV1, FVC, TLC, DL,CO [12]. However other authors observed significant negative associations between FEV1/FVC, DL,CO/VA and VEGF-D serum concentrations [8,14,22,23]. Additionally Young et al, showed correlation between serum VEGF-D concentration and necessity of supplemental oxygen, air flow obstruction, and bronchial hyperresponsiveness [14].…”

Vitamin D binding protein: a new biomarker of disease severity in lymphangioleiomyomatosis

“…In TSC2-deficient models, MMP-2 and MMP-9 are overexpressed in an oestrogen-dependent manner [17,18]. Although serum levels of MMP-2 and MMP-9 do not correlate with lung functions, MMP-9 expression in LAM biopsy specimens correlates with pulmonary function parameters, including FEV1 and diffusing capacity of the lung for carbon monoxide [19,20]. Vitamin D can inhibit the conversion of metalloproteinases including MMP-2 and MMP-9 to their active forms in lung fibroblasts [21].…”

“…An important caveat is that serum VTDB levels were not associated with all prospective changes in lung function in either the NHLBI or the UK cohort. If validated in other cohorts, one might envision a time when these and other biomarkers of disease activity and progression (including VEGF-D, menopausal status and other factors) [19] could be combined into a "LAM Progression Score", with critical implications for personalised therapeutic decision-making and clinical trial design.…”