Seronegative antiphospholipid syndrome: refining the value of “non-criteria” antibodies for diagnosis and clinical management

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombotic manifestations and/or pregnancy-related complications in patients with persistently high antiphospholipid antibodies (aPL), the most common being represented by anticardiolipin antibodies...

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Vydáno v:Haematologica (Roma) Ročník 105; číslo 3; s. 562 - 572
Hlavní autoři: Pignatelli, Pasquale, Ettorre, Evaristo, Menichelli, Danilo, Pani, Arianna, Violi, Francesco, Pastori, Daniele
Médium: Journal Article
Jazyk:angličtina
Vydáno: Italy Ferrata Storti Foundation 01.03.2020
Témata:
Antibodies, Anticardiolipin
Antibodies, Antiphospholipid
Antiphospholipid Syndrome - diagnosis
Antiphospholipid Syndrome - drug therapy
beta 2-Glycoprotein I
Female
Humans
Lupus Coagulation Inhibitor
Pregnancy
Review
ISSN:0390-6078, 1592-8721, 1592-8721
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Shrnutí:Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombotic manifestations and/or pregnancy-related complications in patients with persistently high antiphospholipid antibodies (aPL), the most common being represented by anticardiolipin antibodies (aCL), anti-beta 2 glycoprotein-I (aβ2GPI), and lupus anticoagulant (LAC). A growing number of studies have showed that, in some cases, patients may present with clinical features of APS but with temporary positive or persistently negative titers of aPL. For these patients, the definition of seronegative APS (SN-APS) has been proposed. Nevertheless, the negativity to classic aPL criteria does not imply that other antibodies may be present or involved in the onset of thrombosis. The diagnosis of SN-APS is usually made by exclusion, but its recognition is important to adopt the most appropriate anti-thrombotic strategy to reduce the rate of recurrences. This research is in continuous development as the clinical relevance of these antibodies is far from being completely clarified. The most studied antibodies are those against phosphatidylethanolamine, phosphatidic acid, phosphatidylserine, phosphatidylinositol, vimentin/cardiolipin complex, and annexin A5. Moreover, the assays to measure the levels of these antibodies have not yet been standardized. In this review, we will summarize the evidence on the most studied non-criteria aPL, their potential clinical relevance, and the antithrombotic therapeutic strategies available in the setting of APS and SN-APS.
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PP and EE contributed equally to this work.
FV and DP contributed equally to this work as co-senior authors.
ISSN:0390-6078
1592-8721
1592-8721
DOI:10.3324/haematol.2019.221945