24 APS Flashcards
Nonthrombotic clinical features of APS
1) Unexplained thrombocytopenia 2) Livedo reticularis 3) Heart valve disease 4) Renal thrombotic microangiopathy 5) Neurologic manifestation
Subset of APS characterized by widespread thrombotic disease with multiorgan failure and high mortality rate
CAPS
Mc acquired prothrombotic autoimmune disease
APS
___% of children who initially present with APS progress to either have SLE or lupus-like disease
21
Primary APS compared to secondary APS
Younger, arterial thromboses especially cerebrovascular ischemic events
Secondary APS compared to primary APS
Venous thromboembolic events, hematologic, or skin manifestations
___% of children with APS have underlying autoimmune disease
50-60%
T/F Children with JIA have a high rate of APLA but very rarely develop APLA-related thrombotic event
T
APLA Ig subclass that is generally more pathogenic
IgG
Drug that has been shown to block in vitro platelet-activating effects of APLA
HCQ
Mechanisms of APLA that induce fetal loss
Directly binding to trophoblast; inhibit normal invasion of trophoblasts into decidual tissues, causing defective placentation
Mechanism of heparin in preventing fetal loss
Increases trophoblast invasiveness by increasing MMP expression
MC vascular occlusive manifestation of pediatric APS
Venous thrombosis
MC site of venous thrombosis in patients with APS
Deep leg veins
MC reported site of arterial thrombotic event
Ischemic stroke > peripheral arterial thrombosis
