Free full text available in Blood. Diagnostic criteria, clinical features and treatment are covered. Of special interest:
Immunosuppressive therapy has no role, with the exception of catastrophic APS or a concurrent lupus flare as an indication for immunosuppression.
Higher than usual intensity anticoagulation (INR 3.1-4) is no better than standard intensity.
APS is believed to be a state of extreme hypercoagulability. It has been thus widely assumed that patients with APS who have had VTE should undergo lifelong anticoagulation. Unfortunately RCTs have not directly addressed the question. Because evidence from the general population supports indefinite anticoagulation for some patients with idiopathic VTE and patients with APS are at higher risk than the general population, indefinite anticoagulation is widely recommended for secondary VTE prevention in patients with APS.
Future studies may clarify the role of serologic profiling in patients with APS. Preliminary evidence suggests that, for example, patients with the lupus anticoagulant are at higher risk than patients who only exhibit cardiolipin antibodies. Patients who have cardiolipin antibodies, lupus anticoagulant and beta2GPI antibodies (“triple threat”) may have the highest risk.
In patients with VTE due to transient risk factors who are found to have laboratory evidence for APS there is little evidence to guide clinicians, and a finite duration of anticoagulant therapy may be suitable for some patients.
The authors' recommendations for patients with APS and arterial thrombosis differ little from those for the general population (antiplatelet therapy is as good as anticoagulation) although they tend to favor dual antiplatelet therapy over monotherapy. Nuances are discussed in the text.
Obstetric APS is discussed in some detail and not covered here.
Catastrophic APS is mentioned briefly and a detailed review is cited. Specific diagnostic criteria exist and the mainstays of treatment are prompt anticoagulant therapy, corticosteroids (if infection is not present), treatment or elimination of precipitating factors and treatment of an accompanying lupus flare if present.