Antiphospholipid-Syndrom

Ch. Specker

doi:10.1055/s-0037-1618073

Arthritis und Rheuma, Table of Contents

Arthritis und Rheuma 2011; 31(04): 247-256
DOI: 10.1055/s-0037-1618073

Systemischer Lupus erythematodes

Schattauer GmbH

Antiphospholipid-Syndrom

Update zu Diagnose und TherapieThe antiphospholipid syndrome (APS) – diagnosis and treatmentUpdate on diagnosis and therapy

Ch. Specker

¹Klinik für Rheumatologie und Klinische Immunologie, Zentrum für Innere Medizin der Kliniken Essen Süd, Rheumazentrum Rhein-Ruhr, Essen

› Author Affiliations

Abstract

Zusammenfassung

Das Antiphospholipid-Syndrom (APS) ist gekennzeichnet durch eine gesteigerte Thromboseneigung bei serologischem Nachweis erhöhter Antiphospholipid-Antikörper (Anticardiolipin-Antikörper, Antikörper gegen β2Glykoprotein I) oder eines sogenannten Lupus-Antikoagulans. Es findet sich in ca. 20 bis 30 Prozent der Fälle mit systemischem Lupus erythematodes (sekundäres APS), kann aber auch isoliert auftreten (primäres APS). Ursache für die vielfältigen klinischen Manifestationen des APS sind thrombembolische Gefäßverschlüsse venöser oder arterieller Blutgefäße unterschiedlicher Größe und in verschiedenen Organsystemen. Häufigste Manifestationen sind tiefe Beinvenenthrombosen, Lungenembolien und zerebrovaskuläre Ischämien. Durch Obliteration plazentarer Gefäße kommt es auch zu einer gesteigerten Abortneigung, vorzugsweise jenseits der zehnten Schwangerschaftswoche. Ein APS korrekt zu diagnostizieren ist nicht einfach. Nicht jede Thrombose bei Nachweis von Antiphospholipid-Antikörpern ist Ausdruck eines Antiphospholipid-Syndroms und die Abschätzung des Thromboserisikos sowie Art und Intensität einer geeigneten Thromboseprophylaxe sind nach wie vor eine Herausforderung in der Betreuung von APS-Patienten. Eine Immunsuppression ist in der Behandlung des APS nicht wirksam. In dieser Arbeit werden die mehrfach überarbeiteten Klassifikationskriterien des APS, die Labordiagnostik und die aktuellen Empfehlungen zur Therapie dargestellt.

Summary

The antiphospholipid syndrome (APS) is characterized by recurrent arterial or venous thromboembolism or pregnancy loss, in association with antibodies directed against anionic phospholipids or against plasma proteins bound to anionic phospholipids. It occurs in 20 to 30 % of patients suffering from systemic lupus erythematosus (secondary APS) but is also found without underlying connective tissue disease (primary APS). Cause of the huge variety in clinical manifestations is vaso-occlusive disease and not vasculitis in venous or arterial blood vessels of different size and sites (i. e. deep vein thrombosis, pulmonary embolism, cerebro-vascular disease). In accordance to this also fetal abortion, typically beyond the 10^th week of gestation, is caused by infarctions of blood vessels in the placenta. Establishing the correct diagnosis of APS is not easy. Thrombosis might occur due to other reasons even in patients with antiphospholipid antibodies and to estimate the risk of thrombotic complications is challenging, as well as the question if, which, how long and in what strength anticoagulation is recommended. The several times revised classification criteria of APS, issues on laboratory diagnosis and current therapeutic recommendations are discussed in this paper.

Schlüsselwörter

Antiphospholipid-Syndrom - Thrombophilie - Anticardiolipin-Antikörper - Lupus-Antikoagulans - Antikoagulation - systemischer Lupus erythematodes

Keywords

Antiphospholipid syndrome - thromboembolism - anticardiolipin antibodies - lupus anticoagulant - anticoagulation - systemic lupus

Full Text

References

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