Showing papers by "Philippe Lesavre published in 2012"
TL;DR: A critical role of fluid-phase alternative pathway dysregulation in the pathogenesis of C3 glomerulopathies as well as in immune complex-mediated glomerular diseases is suggested.
433 citations
TL;DR: Cysteamine decreases and delays the onset of complications and improves life expectancy in cystinosis, and should be introduced as early as possible during childhood and maintained lifelong.
146 citations
TL;DR: The two salient features of ANCA-associated vasculitis are the restricted microvessel localization and the mechanism of inflammatory damage, independent of vascular immune deposits.
Abstract: The two salient features of ANCA-associated vasculitis (AAV) are the restricted microvessel localization and the mechanism of inflammatory damage, independent of vascular immune deposits. The microvessel localization of the disease is due to the ANCA antigen accessibility, which is restricted to the membrane of neutrophils engaged in β2-integrin–mediated adhesion, while these antigens are cytoplasmic and inaccessible in resting neutrophils. The inflammatory vascular damage is the consequence of maximal proinflammatory responses of neutrophils, which face cumulative stimulations by TNF-α, β2-integrin engagement, C5a, and ANCA by the FcγRII receptor. This results in the premature intravascular explosive release by adherent neutrophils of all of their available weapons, normally designed to kill IgG-opsonized bacteria after migration in infected tissues.
74 citations