Antiphospholipid syndrome in pregnancy: A comprehensive review

Table 1 Candidate criteria for the diagnosis of antiphospholipid syndrome

Clinical domains	Criteria	Points
Macrovascular VTE	VTE with a high-risk VTE profile	1
	VTE without a high-risk VTE profile	3
Macrovascular AT	AT with a high-risk cardiovascular profile	2
	AT without a high-risk cardiovascular profile	4
Microvascular	Suspected (one or more of the following): Livedo racemosa, livedoid vasculopathy, acute/chronic aPL-nephropathy, pulmonary hemorrhage	2
	Established (one or more of the following): Livedoid vasculopathy, acute/chronic aPL-nephropathy, pulmonary hemorrhage, myocardial disease, adrenal hemorrhage	5
Obstetric	Three or more consecutive pre-fetal and/or early fetal demise	1
	Fetal demise in the absence of pre-eclampsia with severe features or PI with severe features	1
	Pre-eclampsia with severe features or PI with severe features with/without fetal demise	3
	Pre-eclampsia with severe features and PI with severe features with/without fetal demise	4
Cardiac	Valve thickening	2
	Valve vegetation	4
Hematology	Thrombocytopenia (20 × 109-130 × 109/L)	2
Laboratory domains
Lupus anticoagulant test	Positive once	1
	Persistent positivity	5
ACL and/or anti-β2GP1	Moderate or high positive (IgM) (aCL and/or anti-β2GP1)	1
	Moderate positive (IgG) (aCL and/or anti-β2GP1)	4
	High positive (IgG) (aCL or anti-β2GP1)	5
	High positive (IgG) (aCL and anti-β2GP1)	7

At least one documented clinical criterion from the listed domains plus a positive antiphospholipid antibody test within three years of diagnosis of the clinical criteria are required for diagnosis. ACL: Anti-cardiolipin antibody; APL: Antiphospholipid antibody; AT: Arterial thrombosis; Ig: Immunoglobulin; PI: Placental insufficiency; VTE: Thromboembolism; β2GP1: β2-glycoprotein-1.