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    Home > Active Ingredient News > Immunology News > First update in 16 years! What is the new classification standard for antiphospholipid syndrome?

    First update in 16 years! What is the new classification standard for antiphospholipid syndrome?

    • Last Update: 2023-01-01
    • Source: Internet
    • Author: User
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    *For medical professionals only, reference


    1999→2006202X?



    Antiphospholipid syndrome (APS) as one of the important causes of acquired thrombophilia and recurrent miscarriage, more and more clinicians pay more and more attention, with the continuous progress of clinical and basic research in the past ten years, the understanding of APS has gradually deepened, 16 years ago APS classification standards can no longer meet the current clinical needs
    。 In recent years, the revised draft of the new APS classification standard, co-led by the American College of Rheumatology (ACR) and the European Union Against Rheumatism (EULAR), has been released, and this ACR Annual Meeting has specially brought early adopter sharing
    .

    From Sapporo to Sydney: What are the limitations of the current APS classification standard? The current APS 2006 Sydney Classification Standard is based on the 1999 Sapporo Standard (Figure 1), which continues to define APS in terms of two dimensions: APS
    clinical criteria and laboratory standards.

    This version of the classification criteria is limited to non-inflammatory thrombotic events and pathological pregnancy for clinical manifestations, and does not involve APLs-related microangiopathy and hematologic damage; In terms of laboratory standards, the lack of a specific positive definition of antiphospholipid antibodies (aPLs) is not clear, risk stratification is not performed, etc
    .
    Therefore, with the methodological progress of standard development, ACR/EULAR took more than four years to release a new APS classification standard
    based on data-driven and expert consultation through four stages (adding content, deleting content, further censoring/matching weights/finalizing thresholds, and instance validation) [1].

    Figure 1.
    The definition of APS [2-3] in the 2006 Sydney Revised Standard
    has been called for: where is the new classification standard that has not yet been published




    Experts first reiterated the difference between classification criteria and diagnostic criteria, and once again emphasized that the APS classification standard was developed this time, focusing on clinical research, and did not consider pharmacoeconomics and clinical treatment decisions, so this classification standard has extremely high specificity (99%) and relatively insufficient
    sensitivity.
    This means that if a patient in the clinic does not meet the classification criteria, it does not mean that it is "definitely not APS"
    .


    Table 1: Differences between classification and diagnostic criteria


    The new classification standard adopts the form of the current popular classification standard point system, focuses on modifying the definition of the main clinical features, and comprehensively and deeply expands and defines the six clinical main signs (Domains) of APS, namely: D1 - venous event, D2 - arterial event, D3 - microangiopathy, D4 - pathological pregnancy, D5 - heart valve disease, D6 - blood system damage, D7 - LA test and D8 - aCL/aβ2GP1 test


    Proposed update: D1 & D2 macrovascular lesions


    Update D1 "Yes/no venous thromboembolism (VTE) with risk factors" and D2 "Whether arterial thrombosis (AT) is associated with high risk factors for cardiovascular disease (CVD)" to clarify the risk stratification of traditional thrombotic risk factors for macrovascular events, as follows:

    • Main risk factors for VTE (1 or more are high-risk) - active malignancy, hospitalization, severe trauma, high-risk surgery;


    • Secondary risk factors for VTE (two or more high-risk) – active systemic autoimmune disease or active inflammatory bowel disease, acute/active severe infection, central venous catheter, hormone replacement therapy (estrogen-containing oral contraceptives or ongoing IVF pregnancy), long journey, obesity, pregnancy or postpartum pregnancy, prolonged immobilization or low-risk surgery;
    • CVD risk factors (1 or more are high risk) - severe hypertension, long-term diabetes, severe hyperlipidemia, chronic kidney disease;
    • Moderate risk factors for CVD (3 or more are high risk) – no severe hypertension, diabetes, moderate hyperlipidemia, current smoking, obesity;



    Proposed update: D3 microangiopathy


    Updated the content of D3 "Suspected microangiopathy" and "Confirmed microangiopathy", and clarified the definition of microangiopathy, which is detailed as follows:


    • Suspicious microangiopathy – livedo reticularis (signs), bruistry angiopathy (signs), acute/chronic aPLs-related nephropathy (signs or laboratory tests), alveolar hemorrhage (symptoms and images);


    • Confirmed microangiopathy – erythematous angiopathy (signs and pathology), acute/chronic aPLs-associated nephropathy (pathology), myocardial pathology (imaging and pathology), adrenal bleeding (imaging and pathology);




    Editor's note:


     aPLs-associated nephropathy (APLN) is a disease in which aPLs coexist with histopathological changes, in which thrombotic microangiopathy involving arterioles and glomerular capillaries is seen, and can be combined with one or more other manifestations (fibroinal hyperplasia involving organic thrombus, with or without recanalization; Occlusion of arterial and arteriole fibers and/or fibroblasts; focal cortical atrophy; Tubular thyroidization, i.
    e.
    , large atrophic tubular areas containing eosinophilic casts).


    Proposed update: D4 pathological pregnancy


    Updated the content of D3 pathological pregnancy to redefine pathological pregnancy, as follows:


    • 3 or more consecutive pre-embryonic/embryonic deaths (within 10 weeks' gestation) and/or fetal deaths (10 weeks, 0 days to 15 weeks, 6 days);


    • Fetal death (16 weeks 0 days gestation - 33 weeks 6 days) without severe manifestations of pre-eclampsia or placental insufficiency;


    • preeclampsia or placental insufficiency with or without fetal death (severe manifestations of one of the complications, within 34 weeks and 0 days of pregnancy);


    • Preeclampsia or placental insufficiency with and without fetal death (severe manifestations of complications are seen, within 34 weeks and 0 days of pregnancy).




    Editor's note:

    Only an empty or yolk sac is preembryonic death, an embryo is detectable and embryonic smaller than normal size within 10 weeks is embryonic death, a head-rump length of more than 30 mm or other measurements (e.
    g.
    , double parietal diameter measurement) consistent with a stillbirth over 10 weeks of pregnancy is considered fetal death
    .


    Proposed update: D5 heart valve lesion and D6 blood system damage


    Added content on D5 valvular lesions "Valve thickening", "Valve vegetations" and D6 blood system damage "Thrombocytopenia (minimum 20-130x109/L)" to increase the focus on APLs-related valvular heart lesions and thrombocytopenia, among which:


    Table 2: Specificity of varying degrees of thrombocytopenia for APS


    To be updated: D7 - LA test


    Updated the coagulation assay-based LA tests "single positive" and "persistent positive" to emphasize single, double, and triple aPL positivity
    based on different definitions and weights.


    Proposed update: D8——aCL/ aβ2GPI test


    Update the content of aCL/aβ2GPI detection based on ELISA method, distinguish aCL/aβ2GPI-positive patients according to IgM and lgG, so as to guide the setting of clinical trial inclusion criteria, and define two clinically relevant aCL/aβ2GPI positive thresholds, as follows:


    IgM type aCL and/or aβ2GPI are moderately high positive


    IgG type aCL and/or aβ2GPI are moderately positive


    IgG type aCL or aβ2GPI is highly positive


    IgG type aCL and aβ2GPI are highly positive


    The threshold for ELISA method is 40-79 U for medium titers and high titers for 80 U or greater


    summary


    After more than 4 years, the revised APS classification standard has redefined the clinical main characteristics of APS, so that everyone has a new understanding of APS and further broadened the vision
    of clinicians.

    However, there are still some regrets, such as the taxonomic criteria focusing too much on specificity and losing sensitivity; The clinical definition of pathologic pregnancy is too strict; Novel antiphospholipid antibodies, including antiphosphatidylserine prothrombin complex, were not included; Failure to accommodate acceptance of novel antiphospholipid antibody detection methods (chemiluminescence).


    The diagnostic efficacy of this classification standard in the general population, as well as the elderly, children and other special groups, needs to be further confirmed
    .
    Expert profiles




    Professor Zhao Jiuliang



    Bo Shi, Associate Professor, Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Master Supervisor, Assistant to Director

    Mainly engaged in basic and clinical research
    on rheumatic immune diseases such as systemic lupus erythematosus, antiphospholipid syndrome, and pulmonary hypertension associated with connective tissue disease.
    He has successively participated in the national "Eleventh Five-Year Plan", "Twelfth Five-Year Plan" and "Thirteenth Five-Year" scientific and technological support plan rheumatology and immunology related topics

    He has spoken 19 times at national academic conferences and 9 international conferences on behalf of the team, and participated in the publication of more than 106 SCI articles, including the first author and corresponding author SCI 28 papers, won the International Youth Award of the Japanese Society of Rheumatology, the Youth Award of the East Asian Society of Rheumatology, the Travel Award of the International Lupus Conference, the Outstanding Paper Award of the Rheumatology Society of the Chinese Medical Association, the "Golden Pen Award" of the Chinese Medical Journal, the "Top Ten Young Investigators Award" of the 2020 China Medical Doctor Daily, and the 2nd Xiehe Outstanding Youth Award

    At present, he is the vice chairman of the Youth Committee of the Rheumatology Branch of the Chinese Medical Association, the vice chairman of the Youth Committee of the Asia-Pacific Rheumatology Alliance (APLAR), the vice chairman of the Youth Committee of the Rheumatology and Immunologist Branch of the Chinese Medical Doctor Association, the standing committee member and secretary general of the Rheumatology and Immunology Professional Committee of the Chinese Research Hospital Association, the member and secretary of the Beijing Rheumatology Society, and the "Frontiers in" Associate Editor of Medicine (IF 5.
    095),
    Corresponding Editorial Board Member of Chinese Medical Journal, Young Editorial Board Member
    of Union Medical Journal and Chinese Journal of Clinical Immunology and Allergology.


    References:

    [1]Barbhaiya M, Zuily S, Ahmadzadeh Y, et al.
    Development of a new international antiphospholipid syndrome classification criteria phase I/II report: generation and reduction of cand
    idate criteria[J].
    Arthritis care & research, 2021, 73(10): 1490-1501.

    [2] Miyakis S, Lockshin M D, Atsumi T, et al.
    International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)[J].
    Journal of thrombosis and haemostasis, 2006, 4(2): 295-306.

    [3] Wilson W A, Gharavi A E, Koike T, et al.
    International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop[J].
    Arthritis & Rheumatism: Official Journal of the American College of Rheumatology, 1999, 42(7): 1309-1311.


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