Cofactor-Independent Antiphospholipid Antibodies: Implications for Pathogenesis, Diagnosis, and Treatment of Antiphospholipid Syndrome

Abstract

The antiphospholipid syndrome (APS) has occupied haemostaseologists, rheumatologists and obstetricians since its initial description 35 years ago. Its name has been coined because of the antibodies against phospholipids which were the common property of affected patients. In particular, the pathogenesis of APS has been intensively studied after the early discovery that it was possible to induce the clinical manifestations in animals by transfer of antiphospholipid antibodies (aPL). In recent years, it has become clear that aPL are not only structurally heterogeneous but also have different pathogenic properties. This review will focus on the relevance of antigenic specificity of aPL in terms of pathogenesis, diagnosis, and perhaps treatment of APS.

Zusammenfassung

Das Antiphospholipidsyndrom (APS) beschäftigt Hämostaseologen, Rheumatologen und Geburtshelfer seit seiner ersten Beschreibung vor 35 Jahren. Sein Name wurde durch die Antikörper gegen Phospholipide geprägt, die bei den betroffenen Patienten gefunden wurden. Insbesondere nachdem frühzeitig erkannt worden war, dass die klinischen Manifestationen bei Tieren durch Übertragung von Antiphospholipid Antikörpern (aPL) induziert werden können, wurde die Pathogenese des APS intensiv untersucht. In den letzten Jahren wurde deutlich, dass aPL nicht nur strukturell heterogen sind, sondern auch unterschiedliche pathogene Eigenschaften aufweisen. Diese Übersichtsarbeit konzentriert sich auf die Relevanz der Antigenspezifität von aPL in Bezug auf Pathogenese, Diagnose und möglicherweise Behandlung von APS.

Keywords

antiphospholipid antibodies - antiphospholipid syndrome - β2-glycoprotein I - lupus anticoagulant

Schlüsselwörter

Antiphospholipid-Antikörper - Antiphospholipid-Syndrom - β2-Glycoprotein I - Lupus Antikoagulanz

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