Update on antiphospholipid antibody syndrome

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occur in patients without any other autoimmune disease. Despite the autoimmune nature of this syndrome, APS treatment is still based on anticoagulation and antiplatelet therapy.

Development of severe thrombocytopenia 3 months after delivery complicated with early-onset preeclampsia: a case report

“…There is substantial evidence that aPL is associated with recurrent late pregnancy loss, early-onset preeclampsia, placental abruption, and fetal growth restriction. 1) Several mechanisms have been proposed regarding the presence of aPL in association with obstetric clinical manifestations. aPL reportedly causes endothelial and monocyte activations, which lead to thrombotic state in various tissues.…”

“…However, other studies could not demonstrate the association between aPL and preeclampsia and thus concluded that routine screening is not indicated for women with a history of preeclampsia. 1) Moreover, the revised JSSHP guidelines do not stipulate that women with early-onset preeclampsia should be tested for aPL after delivery. 3) Yet, the gestational stage when preeclampsia develops, and clinical findings of preeclampsia, may differ between women with aPL and those without aPL.…”

“…Antiphospholipid syndrome (APS) increases the risk of recurrent pregnancy loss, still birth, and ischemic placental dysfunction such as severe preeclampsia and fetal growth restriction. 1) Women with a history of eclampsia or early-onset preeclampsia should be tested for antiphospholipid antibodies (aPL). However, most women with preeclampsia show improvement in clinical findings as well as laboratory test results within 3 months after delivery.…”

Purpura fulminans–like lesions in antiphospholipid syndrome with endothelial C3 deposition

“…APS can be either primary or secondary to autoimmune diseases, mainly to systemic lupus erythematosus, but it can also be associated with other autoimmune, malignant, or drug-induced diseases. 1 …”

Assinatura de interferon tipo I na síndrome antifosfolípide primária

“…Como mencionado anteriormente, a SAF é uma vasculopatia mediada por autoanticorpos. As tromboses e os eventos gestacionais são suas principais manifestações clínicas 1,2 . A presença de anticorpos antifosfolípides, embora relevante para confirmar o diagnóstico, parece não ser suficiente para explicar completamente a fisiopatologia da doença e um segundo gatilho é geralmente necessário 9 .…”