Methacholine reactivity in lymphangioleiomyomatosis is inversely related to FEV1 and VEGF-D

Lymphangioleiomyomatosis (LAM) is a multisystem disease characterised by cystic lung destruction, leading to respiratory failure, and associated with kidney (e.g. angiomyolipomas (AML)) and lymphatic involvement (e.g. lymphangioleiomyomas, chylous effusions) [1, 2]. LAM occurs sporadically or in association with tuberous sclerosis complex (TSC), an autosomal-dominant disorder characterised by mutations of the TSC1 or TSC2 genes. Lung destruction results from the proliferation of LAM cells, which possess neoplastic properties and are found in LAM lung nodules, in association with fibroblasts, mast cells, lymphocytes and lymphatic endothelial cells [3, 4]. LAM patients may show increases in serum levels of the lymphangiogenic factor, vascular endothelial growth factor-D (VEGF-D), a LAM biomarker used in differential diagnosis of cystic lung diseases and to identify LAM patients likely to respond to sirolimus treatment [5–7].

Source: European Respiratory Journal - June 17, 2021 Category: Respiratory Medicine Authors: Cassandro, R., Elia, D., Caminati, A., Pacheco-Rodriguez, G., Stylianou, M., Moss, J., Harari, S. Tags: Original Articles: Research letters Source Type: research