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Antiphospholipid Antibody Syndrome: From Bench to Bedside by Pier Luigi Meroni

By Pier Luigi Meroni

This ebook, a part of the sequence infrequent illnesses of the Immune approach, deals complete, updated assurance of the pathophysiology and administration of the antiphospholipid syndrome (APS). Immunologic and genetic elements are mentioned and the pathogenic mechanisms accountable for such phenomena as APS-mediated thrombosis and being pregnant loss/complications are defined. the most scientific manifestations, class standards and diagnostic instruments are pointed out, and shut recognition is paid to the character of the involvement of assorted organs or organ platforms in APS. particular chapters describe the remedy of the several indicators, cures of price in keeping off recurrences, and leading edge remedy ways. The authors are senior specialists within the box who're aided by means of more youthful fellows, making sure that the publication can also be educationally orientated. this convenient quantity should be a beneficial instrument for postgraduates in education and execs wishing to increase their wisdom of this particular syndrome.

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In spite of the experimental results, the role of inflammation in determining APS pregnancy complications still remains a debated issue. In APS placentas a strong infiltration of complement components, as well as histopathological findings of deciduitis and villous infiltration, has been found more frequently compared to normal controls [42–45]. On the contrary, further ex vivo examinations of APS placentas and subsequent in vivo studies, using another model of fetal resorption and growth retardation, injected with small amount of human aPL IgG (10–50 μg/ mouse) before implantation, failed to show any sign of acute placental 4 The Pathogenic Mechanisms for Antiphospholipid Antibodies (aPL) in pregnancy loss 41 inflammatory events and complement deposition [46].

These changes facilitate a regular supply of maternal blood to the developing placenta at an optimal flow rate necessary for the adequate delivery of nutrients to the developing embryo [13–16]. Noteworthy, VTC exposed to maternal blood circulating in the intervillous space and EVTC present in human decidua, being of fetal origin, represent a real challenge for the maternal immune system. As a result, a vigorous response may be mounted against paternal antigens expressed on trophoblasts leading to potential deleterious effects on fetal survival [17].

2 Pathogenic clotting mechanisms mediated by aPL. aPL actions favor clot formation through several routes. 1 aPL interact with endothelial cells, primarily through binding of β2GPI on the cell surface, and induce a procoagulant and proinflammatory endothelial phenotype. 2 aPL upregulate tissue factor expression on endothelial cells and blood monocytes, and promote endothelial leukocyte adhesion, cytokine secretion, and PGE2synthesis. 3 aPL recognize phospholipidbinding proteins expressed on platelets—aPL binding potentiates platelet aggregation induced by another agonist.

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