Table 3.

Summary of treatment recommendations for APS and APLA.

Clinical setting	Recommendation	Additional comments
First venous thrombosis in APS	Anticoagulation with VKA (INR 2–3)	No clear benefit of high intensity anticoagulation
		Insufficient data to support routine DOAC use
Arterial thrombosis in APS	ASA + standard intensity anticoagulation with VKA (INR 2–3), or	One study showed no benefit of ASA+VKA over ASA in APS with stroke; results are not generalizable since APLA tested only at baseline and lower target INR
	High intensity VKA anticoagulation (INR 3–4) in high risk patients
Recurrent thrombosis in APS	Confirm that therapeutic INR is maintained	No clinical trial data. Based on clinical observation and expert opinion.
	May consider LMWH or high intensity anticoagulation with VKA (INR 3–4)..
	Consider adjunctive hydroxychloroquine, statin
Obstetric APS (without prior thrombosis)	ASA + LMWH
	Prophylactic dose LMWH continued until 6 weeks post-partum
Obstetric APS with prior thrombosis	Low dose aspirin with therapeutic dosing of LMWH.	Start LMWH (and stop VKA) at or prior to diagnosis of pregnancy.
	After pregnancy, may be transitioned back to VKA to continue anticoagulation indefinitely.
Catastrophic APS	UFH plus high dose steroids (methylprednisolone 1000 mg/kg/day for 3 or more days)	Observational data supports the use of eculizumab, rituximab, plasmapheresis and defibrotide.
Asymptomatic APLA	Consider aspirin for patients with other cardiovascular risk factors	No role of primary prophylaxis with aspirin in the absence of cardiovascular risk factors
	Hydroxychloroquine for patients with concomitant SLE
	Thromboproplylaxis in high risk situations such as surgery or hospitalization
	Address reversible risk factors such as obesity, smoking, combined oral contraceptives