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

By Pier Luigi Meroni

This e-book, a part of the sequence infrequent illnesses of the Immune process, deals finished, up to date insurance of the pathophysiology and administration of the antiphospholipid syndrome (APS). Immunologic and genetic facets are mentioned and the pathogenic mechanisms answerable for such phenomena as APS-mediated thrombosis and being pregnant loss/complications are defined. the most medical manifestations, class standards and diagnostic instruments are pointed out, and shut consciousness is paid to the character of the involvement of assorted organs or organ platforms in APS. particular chapters describe the therapy of the various signs, cures of price in heading off recurrences, and leading edge remedy ways. The authors are senior specialists within the box who're aided by way of more youthful fellows, making sure that the ebook can also be educationally orientated. this convenient quantity may be a worthwhile instrument for postgraduates in education and execs wishing to increase their wisdom of this particular syndrome.

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An important feature of the decidual tissue in the early phase of pregnancy is represented by the active angiogenic differentiation of endometrial endothelial cells (HEECs) and the vascular remodeling of the spiral arteries [13]. In particular decidualizing endometrial cells produce critical angiogenic molecules, such as vascular endothelial growth factor (VEGF) able to promote the angiogenic differentiation of HEECs [14–16]. 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].

Blood 105:1540–1545 Thiagarajan P, Shapiro SS (1998) Lupus anticoagulants and antiphospholipid antibodies. 1 Introduction Arterial and/or venous thrombosis in association with the persistent presence of antiphospholipid antibodies (aPL) is the hallmark of the antiphospholipid antibody syndrome (APS). aPL can be detected by solid-phase assays (anticardiolipin [aCL] and/or anti-β2 glycoprotein I [β2GPI]) and/or by a functional assay (lupus anticoagulant [LA]) [1]. Besides their diagnostic value, aPL are also pathogenic autoantibodies that mediate the vascular manifestations in association with an additional second hit (two hits theory) [2].

It is important to underline that only some antibodies to a specific domain of β2GPI express LAC activity and correlate strongly with thromboembolic events [34]. Other autoantibodies to β2GPI may not be pathogenic, and this may explain why studies on aβ2GPI antibody detection in solid-phase assays do not produce uniform results as IgG aβ2GPI is associated with thrombosis in only a subset of patients. In those cases (from 2 to 10 %) in which aβ2GPI is the sole antibody detected in patients with clinical manifestations of APS, it may not be pathogenic.

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