Phospholipid inhibitors

Summary

The antiphospholipid syndrome (APS) is defined by the association of arterial and/or venous thrombosis and/or pregnancy complications with the presence of at least one among the main antiphospholipid antibodies (aPL) (i. e., Lupus anticoagulants, LA, IgG and/ or IgM anticardiolipin antibodies, aCL, IgG and/or IgM antiβ2-glycoprotein I antibodies, aβ2-GPI). Several clinical studies have consistently reported that LA is a stronger risk factor for both arterial and venous thrombosis compared to aCL and aβ2-GPI. In particular, LA activity dependent on the first domain of β2-GPI and triple aPL positivity are associated with the risk of thrombosis and obstetrical complications.

Asymptomatic aPL-positive subjects do not require primary thromboprophylaxis. Venous thromboembolism is the most common initial clinical manifestation of APS. To prevent its recurrence indefinite anticoagulation is recommended. Long duration treatment with warfarin or aspirin is used after a first cerebral arterial thrombosis. Low molecular weight heparin (LMWH) with or without aspirin is recommended to reduce the rate of obstetrical complications of APS pregnant women.

Zusammenfassung

Das Antiphospholipid-Syndrom (APS) ist definiert als die Kombination aus arteriellen und/ oder venösen Thrombosen und/oder Schwangerschaftskomplikationen und Vorhandensein von mindestens einem der wichtigsten Antiphospholipid-Antikörper (aPL), d. h. Lupus-Antikoagulans (LA) IgG-und/oder IgM-Anti-Cardiolipin-Antikörper (aCL), IgG-und/oder IgMAnti-β2-Glykoprotein-I-Antikörper (aβ2-GPI). Verschiedene klinische Studien ergaben übereinstimmend, dass LA verglichen mit aCL und aβ2-GPI der stärkere Risikofaktor für arterielle sowie venöse Thrombosen ist. Insbesondere sind die von Domäne I des β2-GPI-abhängige LA-Aktivität sowie dreifach positive aPL mit dem Risiko für Thrombosen und geburtshilfliche Komplikationen assoziiert.

Asymptomatische aPL-positive Patienten benötigen keine primäre Thromboseprophylaxe. Venöse Thromboembolien sind die häufigste klinische Erstmanifestation des APS. Zur Rezidivprophylaxe wird eine zeitlich unbegrenzte Antikoagulation empfohlen. Nach einer ersten zerebralen arteriellen Thrombose erfolgt die Langzeitbehandlung mit Warfarin oder ASS. Niedermolekulares Heparin (LMWH) mit oder ohne ASS wird zur Reduktion der geburtshilflichen Komplikationsrate bei schwangeren APS-Patientinnen empfohlen.

Keywords

Antiphospholipid syndrome - thrombosis - abortion - antiphospholipid antibodies

Schlüsselwörter

Antiphospholipid-Syndrom - Thrombose - Fehlgeburt - Antiphospholipid-Antikörper

• References

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