Abstract
Antiphospholipid syndrome (APS) is characterized by thrombotic events and obstetric complications in the presence of persistently positive antiphospholipid antibodies. Obstetric manifestations include, recurrent miscarriages, fetal death at or beyond the 10th week of gestation, and premature birth due to pre-eclampsia/placental insufficiency. Even now, both clinical features and laboratory parameters are controversial. Both can be used to stratify women with APS in terms of risk of adverse pregnancy outcome, and thus adjust treatment. APS pregnancies should be classified into low, medium and high-risk classes based on clinical and laboratory features. Depending on the risk class, the most appropriate therapy must be then selected. Heparin plus LDA is considered the standard of care for patients with a confirmed diagnosis of obstetric APS and generally results in over 70–80% successful pregnancies. The 20–30% pregnancies in which treatment fails are defined as “high-risk” or “refractory” pregnancies. Numerous treatments have been used in addition to standard of care, to treat these patients, but no well-designed trial has yet been conducted. New insights into the etiopathogenetic mechanisms of obstetric APS have led to the testing of new therapeutic approaches, that may soon change the way we manage this condition.
Highlights
Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease characterized by thrombotic events and obstetric complications in the presence of persistently positive antiphospholipid antibodies [1]
The findings from this review suggest introducing low-dose Intravenous immunoglobulins (IVIG) (< 2 g/kg/month) or HCQ 400 mg/day before pregnancy in women with APS refractory to conventional therapy, and high-dose IVIG (2 g/kg/month) in combination with plasma exchange (PE) or alone in those with high risk/refractory APS with both approaches leading to improved pregnancy outcome
Contrary to what was first thought, aPLs determine pregnancy morbidity with both inflammatory and non-inflammatory mechanisms. These findings have led to a better understanding of the different features of obstetric APS
Summary
Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease characterized by thrombotic events and obstetric complications in the presence of persistently positive antiphospholipid antibodies (aPLs) [1]. The classification criteria (Table 1), developed in 1999 [2] and revised in 2006 [1] include clinical features consisting of thrombosis and/or obstetric morbidity in the presence of laboratory criteria such as lupus anticoagulant (LA), medium-high titer IgG/IgM anticardiolipin antibodies (aCL) and/or anti-β2 glycoprotein I (anti-β2GPI). They are often used, as diagnostic tools. Management of OAPS is challenging for the physician, as individual women with APS do not have the same obstetric risk profile. This chapter aims to clarify aspects of pathogenesis, clinical features, risk stratification and therapeutic strategies in OAPS
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