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Rheumatology

APS

Classification

2023 ACR/EULAR antiphospholipid syndrome classification criteria

2023 ACR/EULAR antiphospholipid syndrome classification criteria


Entry criteria

At least one documented clinical criterion listed below (domains 1-6)
PLUS
A positive antiphospholipid antibody test (a lupus anticoagulant test, or moderate-to-high titers of anticardiolipin or anti-beta2-glycoprotein-1 antibodies IgG or IgM) within three years of the clinical criterion
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Clinical domains

D1: Macrovascular (Venous thromboembolism [VTE])

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D2: Macrovascular (Arterial Thrombosis [AT])

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D3: Microvascular
Suspected (one or more of the following)
Livedo racemosa (exam)
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Livedoid vasculopathy lesions (exam)
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Acute/chronic aPL-nephropathy (exam or lab)
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Pulmonary hemorrhage (symptoms and imaging)
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Established (one of more of the following)
Livedoid vasculopathy (pathology)
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Acute/chronic aPL-nephropathy (pathology)
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Pulmonary hemorrhage (BAL or pathology)
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Pulmonary hemorrhage (BAL or pathology)
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Myocardial disease (imaging or pathology)
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Adrenal hemorrhage (imaging or pathology)
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D4 Obstetric
≥3 Consecutive pre-fetal (<10w) and/or early fetal (10w 0d -15w 6d) death
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Fetal death (16w 0d - 33w 6d) in the absence of pre-eclampsia (PEC) with severe features or placental insufficiency (PI) with severe features
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PEC with severe features (<34w 0d) or PI with severe features (<34w 0d) with/without fetal death
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PEC with severe features (<34w 0d) and PI with severe features (<34w 0d) with/without fetal death
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D5: Cardiac valve
Thickening
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Vegetation
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D6 Haematology
Thrombocytopenia (lowest 20-130x10^9/L.)
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Laboratory (aPL) domains

Anticardiolipin antibodies (aCL) IgM

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Anticardiolipin antibodies (aCL) IgG

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Anti-β2-glycoprotein-1 (anti-βGP1) IgM

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Anti-β2-glycoprotein-1 (anti-βGP1) IgG

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Lupus anticoagulant (LAC)

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Statistics

Sensitivity: 84%

Specificity: 99%

Considerations

The tool is for educational use only, is not meant to be a substitute for professional advice and should not be used for medical diagnosis and/or medical treatment.

Classification criteria are designed for research purposes.

Reference

Barbhaiya M, Zuily S, Naden R, Hendry A, Manneville F, Amigo MC, Amoura Z, Andrade D, Andreoli L, Artim-Esen B, Atsumi T, Avcin T, Belmont MH, Bertolaccini ML, Branch DW, Carvalheiras G, Casini A, Cervera R, Cohen H, Costedoat-Chalumeau N, Crowther M, de Jesus G, Delluc A, Desai S, De Sancho M, Devreese KM, Diz-Kucukkaya R, Duarte-Garcia A, Frances C, Garcia D, Gris JC, Jordan N, Leaf RK, Kello N, Knight JS, Laskin C, Lee AI, Legault K, Levine SR, Levy RA, Limper M, Lockshin MD, Mayer-Pickel K, Musial J, Meroni PL, Orsolini G, Ortel TL, Pengo V, Petri M, Pons-Estel G, Gomez-Puerta JA, Raimboug Q, Roubey R, Sanna G, Seshan SV, Sciascia S, Tektonidou MG, Tincani A, Wahl D, Willis R, Yelnik C, Zuily C, Guillemin F, Costenbader K, Erkan D; ACR/EULAR APS Classification Criteria Collaborators. 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria. Arthritis Rheumatol. 2023 Aug 28. doi: 10.1002/art.42624. Epub ahead of print. PMID: 37635643.

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