The emerging role of multiple antiphospholipid antibodies positivity in patients with antiphospholipid syndrome

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by clinical symptoms of vascular thrombosis and/or pregnancy morbidity in the presence of autoimmune antiphospholipid antibodies (aPL). Current laboratory APS criteria include the presence of at least one of the three relevant aPL: lupus anticoagulant, anticardiolipin antibodies and anti-β₂ glycoprotein I antibodies. Therefore, patients could have a single aPL pattern or combinations of aPL. Evidence arising from clinical experience indicates that patients having the highest aPL titer and simultaneous aPL detected by different tests have a worse prognosis and a higher probability of recurrence of the APS clinical features. In recent years, an emerging role of multiple aPL positivity in the identification of high-risk patients with aPL/APS is evident. This paper will review the current knowledge on the clinical relevance of having single or multiple aPL positivity.

Keywords:

• antiphospholipid antibodies
• antiphospholipid syndrome
• arterial thrombosis
• pregnancy morbidity
• venous thrombosis

Financial & competing interests disclosure

The authors are supported by a grant from the National Fund of Science and Technology (PICT 2010-1173), Ministry of Culture and Education, Argentina. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Key issues

• The antiphospholipid syndrome (APS) is an autoimmune disease characterized clinically by the occurrence of either venous or arterial thrombosis in different vascular beds, and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPL).

• The positivity for at least one recommended aPL test: lupus anticoagulant and/or IgG/IgM anticardiolipin and/or anti-β₂ glycoprotein I antibodies are part of the current laboratory APS criteria.

• There is no enough clinical evidence that the measurement of other members of the aPL family could be essential in the APS diagnosis.

• At the present time, the measurement of aPS/PT and anti-domain I anti-β₂ glycoprotein I antibodies might be useful in assessing the thrombotic risk in APS patients.

• Evidence arising from clinical experience indicates that patients having the highest aPL titer and simultaneously aPL detected by different tests have a worse prognosis and a higher probability of recurrence of the APS clinical features.

• The risk of thrombosis increases with the number of positive tests in APS patients and also in asymptomatic carriers of persistent aPL.

• The definition of APS should include a risk stratification to develop APS-related events according to the aPL profile.

• Patients with primary or systemic lupus erythematosus-associated APS and multiple aPL are at risk of developing future thromboembolic events.