Aiello Anna, Sarti Luca, Sandri Gilda, Poli Daniela, Sivera Piera, Barcellona Doris, Prisco Domenico, Pizzini Attilia Maria, Vercillo Giuseppe, Antonucci Emilia, Palareti Gualtiero, Pengo Vittorio
Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Reggio Emilia, Italy.
Thrombosis Center, Medicina Interna d'Urgenza, Ospedale Civile Baggiovara, Modena, Italy.
Int J Lab Hematol. 2025 Apr;47(2):313-317. doi: 10.1111/ijlh.14416. Epub 2024 Dec 11.
The recently published ACR/EULAR classification criteria score (3 points or more) both clinical and laboratory criteria to define the presence of antiphospholipid syndrome (APS). The clinical criteria have been better defined while laboratory criteria remain the same [lupus anticoagulant (LA), anticardiolipin (aCL) and anti ß2-Glycoprotein I (aß2GPI) antibodies] but with different impact (points) on the classification of patients. APS is excluded if more than 3 years separate positive test for antiphospholipid antibodies (aPL) and clinical manifestation.
The present study evaluates how many patients would be excluded by the new criteria among those enrolled as APS in the START 2 antiphospholipid registry. The analysis includes 380 patients (274 APS and 106 carriers).
Of 274 patients classified as APS, 118 (43%) did not match the new ACR/EULAR criteria for various reasons. First, the determination of aCL and aß2GPI antibodies was performed by automated instrumentations not allowed in the new criteria. Second, laboratory test score was less than 3 and this was due to an isolated IgM aCL or IgM aß2GPI in most cases and to isolated LA unconfirmed after 12 weeks in few cases. Third, 2 patients had a positive laboratory tests more than 3 years after the clinical event. Of the 106 carriers, 62% had aCL and aß2GPI determined by ELISA thus meeting the ACL/EULAR laboratory criteria but were negative for clinical criteria.
This study shows that many patients classified as APS in the START 2 registry do not match the classification using the new ACR/EULAR criteria.
最近公布的美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）分类标准通过临床和实验室标准评分（3分或更高）来定义抗磷脂综合征（APS）的存在。临床标准已得到更好的界定，而实验室标准保持不变（狼疮抗凝物[LA]、抗心磷脂[aCL]和抗β2糖蛋白I[aβ2GPI]抗体），但对患者分类的影响（分值）不同。如果抗磷脂抗体（aPL）检测阳性与临床表现相隔超过3年，则排除APS。
本研究评估了在START 2抗磷脂登记处登记为APS的患者中，有多少患者会被新标准排除。分析纳入了380例患者（274例APS患者和106例携带者）。
在274例被分类为APS的患者中，118例（43%）因各种原因不符合新的ACR/EULAR标准。首先，aCL和aβ2GPI抗体的检测是通过新标准不允许的自动化仪器进行的。其次，实验室检测得分低于3分，这在大多数情况下是由于孤立的IgM aCL或IgM aβ2GPI，少数情况下是由于12周后未得到确认的孤立LA。第三，2例患者在临床事件发生3年多后实验室检测呈阳性。在106例携带者中，62%通过酶联免疫吸附测定（ELISA）检测到aCL和aβ2GPI，因此符合ACR/EULAR实验室标准，但临床标准为阴性。
本研究表明，START 2登记处中许多被分类为APS的患者不符合使用新的ACR/EULAR标准的分类。
