Antiphospholipid-Syndrom: neue Klassifikationskriterien und Implikationen für die Praxis

Zusammenfassung

Analog zu anderen entzündlich-rheumatischen Erkrankungen wurden im Oktober 2023 neue hierarchisch gruppierte, gewichtete und risikostratifizierte APS Klassifikationskriterien von der European League Against Rheumatism (EULAR) gemeinsam mit dem American College of Rheumatology (ACR) vorgestellt. Die neuen Kriterien sehen als obligates Eingangskriterium das Vorliegen mindestens einer klinischen APS-Manifestation und eines positiven aPL-Labortests vor (Lupusantikoagulans oder moderat bis deutlich erhöhte IgG- oder IgM-Titer für aCL oder aß2GPI). Die sechs Klinik- und zwei Labordomänen werden mit Werten von 1–7 Punkten gewichtet. Eine Klassifikation als APS kann erfolgen, wenn mindestens jeweils 3 Punkte in den klinischen und den serologischen Domänen erreicht werden. Neu werden traditionelle Thromboserisikofaktoren bei aPL-positiven Personen mitberücksichtigt und eine genauere Charakterisierung aPL-assoziierter mikrovaskulärer, geburtshilflicher und nichtthrombotischer Manifestationen (Thrombozytopenie und Herzklappenbeteiligung) vorgenommen. IgM-aPL zählen als weniger spezifisch und werden mit einer deutlich geringeren Punktezahl (1 Punkt) gegenüber den IgG-aPL (je nach Höhe und Konstellation 4-7 Punkte) berücksichtigt. Die erreichte hohe Spezifität der neuen Klassifikationskriterien wird qualitativ hochwertige Studien im APS mit dem Ziel einer verbesserten Patientenversorgung erlauben. Der Beitrag fokussiert auf aktuelle Herausforderungen durch die Klassifikationskriterien sowie die unabhängig zu sehenden Aspekte der klinischen Diagnosestellung und der daraus erwachsenen Behandlungskonsequenzen.

Abstract

As has been done with other inflammatory rheumatic diseases, new hierarchically grouped, weighted and risk-stratified classification criteria were introduced for Antiphospholipid Syndrome (APS) by the European League Against Rheumatism (EULAR) in collaboration with the American College of Rheumatology (ACR) in October 2023. The new criteria stipulate the mandatory presence of at least one clinical manifestation of APS and a positive aPL laboratory test (lupus anticoagulant or moderately to significantly elevated IgG or IgM titres for aCL or anti-β2GPI) as the entry criteria. The six clinical and two laboratory domains are weighted with values ranging from 1 to 7 points. A classification as APS requires a minimum of 3 points in both the clinical and serological domains. Traditional thrombosis risk factors are now taken into consideration in aPL-positive individuals, and a more detailed characterization of aPL-associated microvascular, obstetric and non-thrombotic clinical manifestations (thrombocytopenia and heart valve involvement) is taken into account. IgM-aPL is considered less specific and is assigned a significantly lower point value (1 point) compared with IgG-aPL (4-7 points depending on the level and constellation). The high specificity associated with the new classification criteria will allow for high-quality studies in APS with the aim of improving patient care. This paper focuses on current challenges posed by the classification criteria as well as aspects to be considered for clinical diagnosis, including treatment consequences.

Schlüsselwörter

APS - Klassifikationskriterien 2023 - Diagnose

Keywords

APS - classification criteria 2023 - diagnosis

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