Zusammenfassung
Das Antiphospholipid-Syndrom (APS) ist eine Autoimmunerkrankung, die charakterisiert wird sowohl durch venöse als auch arterielle Gefäßverschlüsse mit hohem Rezidivrisiko einerseits und einer erhöhten Abortneigung beim so genannten maternalen APS andererseits. Beide Manifestationen der Erkrankung sind mit Antiphospholipid-Antikörpern (APA) in Form des so genannten Lupusantikoagulanz (LA), der Anticardiolipin-Antikörpern (ACA) oder Anti-β2-Glykoprotein-I-Antikörpern (Anti-β2-GPI-Ak) assoziiert. Die pathophysiologische Bedeutung von APA im Rahmen des APS gilt als sicher, wobei die Mechanismen der Thrombosierung bei den beiden klinischen Ausprägungen als multifaktoriell angenommen werden und bislang nicht vollständig verstanden sind. Das Risiko für die Entwicklung einer Thrombose bei positivem APA-Nachweis ohne vorausgegangenes Ereignis von ansonst gesunden Patienten ist leicht erhöht (etwa 1% pro Jahr). Bei vorausgegangenem thrombotischen Ereignis ohne Antikoagulation liegt ein drastisch erhöhtes Thromboserezidivrisiko vor (>10% pro Jahr). Im Jahre 2006 wurden neue Konsensusempfehlungen zur Diagnose des APS empfohlen, die eine solide Basis sowohl für die Praxis als auch für weiterführende Studien darstellen, um noch offene Fragen zur klinischen Führung von APS-Patienten zu beantworten. Bislang vermag eine langfristige prophylaktische Antikoagulation das Rezidivrisiko von Thrombosen zu vermindern. Bei Patientinnen mit APS in der Schwangerschaft kann Heparin mit/ohne ASS fetale und mütterliche Komplikationen signifikant verbessern.
Abstract
Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent vascular thrombosis and loss of pregnancy in association with the presence of antiphospholipid antibodies (APA) detectable as lupus anticoagulants, anticardiolipin antibodies or anti-β2 glycoprotein I antibodies. The pathophysiological importance of APA in APS is accepted, however, the mechanisms leading to thrombosis are likely to be multifactorial and are so far unclear. Without a prior thrombosis, the risk of developing a new thrombosis in healthy patients with APA is slightly increased (<1% per year). However, the risk of a recurrent thrombosis increases considerably (>10% per year) in patients with a history of thrombosis without anticoagulation. The careful and correct identification of patients with APS is important because prophylactic anticoagulation can reduce the risk of recurrent thrombotic events, and during pregnancy can improve fetal and maternal outcome.
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Schuchmann, S., Dörner, T. Antiphospholipid-Syndrom 2007. Z. Rheumatol. 66, 198–205 (2007). https://doi.org/10.1007/s00393-007-0155-7
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DOI: https://doi.org/10.1007/s00393-007-0155-7