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Antiphospholipidsyndrom

Antiphospholipid syndrome

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Zusammenfassung

Das Antiphospholipidsyndrom (APS) stellt mit seinen vielfältigen Manifestationen ein interdisziplinäres Problem dar, dem als gemeinsame Ursache eine gesteigerte Thromboseneigung, aber keine Gefäßentzündung zugrunde liegt. Neben typischen Hautveränderungen (Livedo racemosa) wird das klinische Bild durch venöse oder arterielle Durchblutungsstörungen geprägt, und serologisch sind diese Patienten durch Antiphospholipidantikörper/aPL (Anticardiolipinantikörper/aCL, Lupusantikoagulans/LA) bzw. durch Antikörper gegen Serumproteine gekennzeichnet, die mit anionischen Phospholipiden gekoppelt sind (β2-Glykoprotein I/β2GPI).

Häufigste Komplikationen sind tiefe Beinvenenthrombosen, Lungenembolien und zerebrovaskuläre Ischämien. Durch Obliteration plazentarer Gefäße kommt es zu einer gesteigerten Abortneigung, vorzugsweise jenseits der 10. Schwangerschaftswoche.

Die korrekte Diagnose eines APS zu stellen ist nicht einfach. Die Abschätzung des Thromboserisikos und die Art, Dauer und Intensität einer geeigneten Thromboseprophylaxe sind nach wie vor eine Herausforderung in der Betreuung von APS-Patienten.

Der Leser soll in diesem Beitrag die richtige Interpretation internationaler Klassifikationskriterien des APS und der Labordiagnostik erlernen, um Fallstricke in der Diagnose des APS zu erkennen. Außerdem werden die im konkreten Fall nicht unproblematischen Strategien zur Thromboseprophylaxe erläutert.

Abstract

Antiphospholipid syndrome (APS) is characterized by recurrent arterial or venous thromboembolism or pregnancy loss in association with antibodies directed against anionic phospholipids or plasma proteins bound to anionic phospholipids. A common cause of the huge variety of clinical manifestations is vaso-occlusive disease and not vasculitis in venous or arterial blood vessels of different sizes and sites (i.e. deep vein thrombosis, pulmonary embolism, cerebrovascular disease). In accordance with this, fetal abortion, typically beyond the tenth week of gestation, is also caused by infarctions of blood vessels in the placenta.

Establishing the correct diagnosis of APS is not easy. To estimate the risk of thrombotic complications is challenging, as well as the questions of, which, how long and in what strength anticoagulation is recommended.

This paper should enable the reader to apply international consensus classification criteria correctly, to interpret the different laboratory tests for anti-phospholipid antibodies and to gain an awareness of the different forms of anticoagulation in order to stratify therapeutic decisions.

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  1. Klinik für Rheumatologie & Klinische Immunologie, Katholisches Krankenhaus St. Josef, Zentrum für Innere Medizin der Kliniken Essen Süd, Propsteistraße 2, 45239, Essen, Deutschland

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Specker, C. Antiphospholipidsyndrom. Z Rheumatol 66, 41–53 (2007). https://doi.org/10.1007/s00393-006-0127-3

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