Pathophysiology of the antiphospholipid antibody syndrome

Abstract

Antiphospholipid antibodies (aPL) are associated with the recurrent pregnancy loss and thrombosis that characterizes the antiphospholipid antibody syndrome (APS). Although the ontogeny of these pathogenic antibodies has not been fully elucidated, there is evidence that indicates the involvement of both genetic and environmental factors. The ability of aPL to induce a procoagulant phenotype in APS patients plays a central role in the development of arterial and venous thrombotic manifestations typical of the disease. Inflammation serves as a necessary link between this procoagulant phenotype and actual thrombus development and is an important mediator of the placental injury seen in APS patients with obstetric complications. Recent evidence has indicated a role for abnormal cellular proliferation and differentiation in the pathophysiology of APS, especially in those patients with pregnancy morbidity and other more atypical manifestations that have no identifiable thrombotic cause. The interplay of genetic and environmental factors responsible for aPL development and the mechanisms by which these antibodies produce disease in APS patients is the focus of this review.

Antiphospholipid syndrome masquerading as a case of infective endocarditis

“…CAPS was first described in 1984, affecting about 1% of patients with APS. CAPS is characterised by multiple small vessel occlusions affecting multiple organs 5. Data from the CAPS registry show that pulmonary complications can be found in 24% of cases, neurological complications in 18% and renal complications in 18%.…”

“…A ‘second hit’ hypothesis has been proposed in such cases explained as follows: the presence of antiphospholipid antibodies causes a procoagulant state, this being the ‘first hit’. The ‘second hit’ is the inciting factor, namely infection, inflammation or trauma, which leads to thrombus formation 5. Organisms implicated include a number of pathogens, including Streptococcus .…”

Поражение Почек На Фоне Антифосфолипидного Синдрома

“…К фосфолипид-связывающим плазменным белкам в первую очередь относится β 2 ГПI, а также протромбин, тромбомодулин, кининогены, антитромбин III, белок С, белок S, аннексин I, II и V [20]. Наиболее важным фосфолипидом, с которым связываются эти белки, является фосфатидилсерин, расположенный на внутренней поверхности клеточной мембраны.…”

“…Фосфатидилсерин появляется на поверхности мембран активированных или апоптотических клеток, в том числе трофобластов, моноцитов, эндотелиальных клеток и тромбоцитов [21]. В моноцитах и эндотелиальных клетках антитела против β 2 ГПI могут вызвать активацию тканевого фактора, который играет ключевую роль в инициации внешнего коагуляционного каскада [20].…”

Influence of Secondary Antiphospholipid Syndrome and Secondary Sjögren’s Syndrome on Patient’s Quality of Life with Systemie Lupus Erythematosus

“…Genetic factors and infections may contribute to its development [48,49]. APS is diagnosed on the basis of the modified Sapporo classification criteria [50].…”