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Clinical Question: Does my patient have antiphospholipid syndrome (APLS)?

Item Value
ID 5
Question Does my patient have antiphospholipid syndrome (APLS)?
AlgorithmLink Click here for algorithm
AlgorithmDescription This algorithm is based on the revised Sapporo criteria, which details the precise clinical and laboratory criteria to diagnose antiphispholipid syndrome (J Thromb Haemost. 2006;4(2):295). In brief, the criteria requires vascular thrombosis and/or preganancy-related morbidiy (see reference for full list of clinical criteria) and laboratory criteria. The laboratory tests are beta-2-glycoprotein (aB2GP) IgG or IgM, anti-cardiolipin (aCL) IgG or IgM, and at least two tests for a lupus anticoagulant (at UCSF, a Russel's viper venom and a PTT-based hexogonal phospholipid neutralization are performed). Both aB2GP and aCL are not affected by anti-coagulation medications, but the lupus anticoagulant tests may be. For this reason testing on anti-coagulated blood is ideal, but the particular therepeutic and diagnostic needs of the patient should be taken into account. We do not recommend laboratory testing for antiphospholipid syndrome at the time of an acute qualifying event. The criteria require lab tests to be positive at least 12 weeks after a qualifying event. Moreover, the tests must be repeated a subsequent 12 weeks after the first round of tests.
ICD10 D68.8, I26, O03.9, I63.3, I63.0
PubMedID 18755986, 16420554, 12393574, 22951216

 

Tests associated with this clinical question:

Click on the test name for more information about the use of this test to answer this clinical question

ID Test Code Test Name Comment
22 APLA Anti-Phospholipid Antibody Panel The diagnosis of anti-phospholipid syndrome requires clinical and laboratory evidence (full criteria listed in the revised Sapporo criteria, J Thromb Haemost. 2006;4(2):295). The laboratory evaluation should take place at least 12 weeks after a qualifying clinical event and be repeated at least 12 weeks after the first round of tests. We do not recommend testing for anti-phospholipid syndrome at the time of an acute event. The anti-phospholipid antibody panel includes the most important laboratory tests in this definition: Anti-beta-2-glycoprotein and anti-cardiolipin, IgG and IgM, and two tests for a lupus anticoagulant (Russel's viper venom and a PTT-based hexogonal phospholipid neutralization). The presence of clinical criteria and at least one positive laboratory criteria (with repeat positivity at least 12 weeks apart) establishes a diagnosis of anti-phospholipid syndrome. The battery of tests is very sensitive on initial testing.

Studies that linked anti-phopholipid antibodies and lupus anticoagulants to thrombosis showed varying degrees of association with respect to the various anti-bodies. Presence of a lupus anticoagulant demonstrated the single strongest association. Anti-cardiolipin and anti-beta-2-glycoprotein were more predictive of thrombosis when they were of the IgG type and in high titers. The strongest association was seen when multiple tests were positive. One study suggests prognostic data may be gleaned from the laboratory data: persistently elevated titers of anti-cardiolipin may be a risk factor for thrombotic recurrence in patient's with anti-phospholipid syndrome.

Transient elevation in both anti-cardiolipin IgG and IgM titers occur, frequently in infections. These false positives are more frequent with anti-cardiolipin than anti-beta-2-glycoprotein.

Testing for lupus anticoagulant in the presence of anticoagulant therapy (including warfarin, direct thrombin inhibitors & direct factor 10a inhibitors, and supratherapeutic heparin) is not recommended due to possible interference with test results. The presence of factor deficiencies or a factor specific inhibitor may also interfere with this assay. Clinical correlation is advised.
23 ACLG Anti-Cardiolipin Antibody, IgG In the context of diagnosing anti-phospholipid syndrome, anti-cardiolipin IgG and IgM should be performed in conjunction with other laboratory tests (all pertinent labs are included in the antiphopholipid antibody panel) in patients who meet clinical criteria as defined in the revised Sapporo criteria (J Thromb Haemost. 2006;4(2):295). The laboratory evaluation should take place at least 12 weeks after a qualifying clinical event and be repeated at least 12 weeks after the first round of tests. We do not recommend testing for anti-phospholipid syndrome at the time of an acute event. The presence of clinical criteria and at least one positive laboratory criteria (with repeat positivity at least 12 weeks apart) establishes a diagnosis of antiphospholipid syndrome.

Studies have shown that the association of thrombosis is strenghtened with high titers and IgG subtype of anti-cardiolipin. Persistently elevated titers of anti-cardiolipin may be a risk factor for recurrence in patient's with anti-phospholipid syndrome.

Transient elevation in both anti-cardiolipin IgG and IgM titers occur, frequently in infections. These false positives are more frequent with anti-cardiolipin than anti-beta-2-glycoprotein.

Studies have shown that IgG or IgM anti-cardiolipin demonstrate weaker association with thrombosis than a positive lupus anticoagulant test.
26 HEXA Lupus Anticoagulant by HEXA In the context of diagnosing anti-phospholipid syndrome, a test for a lupus anticoagulant should be performed by at least 2 methods (at UCSF, the two tests are the Russel's viper venom and a PTT-based hexogonal phospholipid neutralization) in conjunction with other laboratory tests (all pertinent labs are included in the anti-phopholipid antibody panel) in patients who meet clinical criteria as defined in the revised Sapporo criteria (J Thromb Haemost. 2006;4(2):295). The laboratory evaluation should take place at least 12 weeks after a qualifying clinical event and be repeated at least 12 weeks after the first round of tests. We do not recommend testing for anti-phospholipid syndrome at the time of an acute event. The presence of clinical criteria and at least one positive laboratory criteria (with repeat positivity at least 12 weeks apart) establishes a diagnosis of anti-phospholipid syndrome.

Studies have shown that the presence of a lupus anticoagulant is more predictive of thrombosis than high titers of anti-cardiolipin or anti-beta-2-glycoprotein.

Testing for lupus anticoagulant in the presence of anticoagulant therapy (including warfarin, direct thrombin inhibitors & direct factor 10a inhibitors, and supratherapeutic heparin) is not recommended due to possible interference with test results. The presence of factor deficiencies or a factor specific inhibitor may also interfere with this assay.
27 PTT Activated Partial Thromboplastin Time Approximately 50% of patients with anti-phospholipid syndrome will have a normal PTT. Therefore, the PTT is not useful in evaluating for antiphospholipid syndrome. The anti-phopholipid antibody panel should be ordered instead. It includes assays for anti-cardiolipin and anti-beta2-glycoprotein (IgG and IgM) and two tests for lupus anticoagulants (Russel's viper venom and a PTT-based hexogonal phospholipid neutralization). The presence of clinical criteria and at least one positive laboratory criteria (with repeat positivity at least 12 weeks apart) establishes a diagnosis of anti-phospholipid syndrome. Laboratory testing must be done at least 12 weeks after a qualifying event.
25 RVVTM Russell's Viper Venom Test In the context of diagnosing anti-phospholipid syndrome, a test for a lupus anticoagulant should be performed by at least 2 methods (at UCSF, the two tests are the Russel's viper venom and a PTT-based hexogonal phospholipid neutralization) in conjunction with other laboratory tests (all pertinent labs are included in the anti-phopholipid antibody panel) in patients who meet clinical criteria as defined in the revised Sapporo criteria (J Thromb Haemost. 2006;4(2):295). The laboratory evaluation should take place at least 12 weeks after a qualifying clinical event and be repeated at least 12 weeks after the first round of tests. We do not recommend testing for anti-phospholipid syndrome at the time of an acute event. The presence of clinical criteria and at least one positive laboratory criteria (with repeat positivity at least 12 weeks apart) establishes a diagnosis of anti-phospholipid syndrome.

Studies have shown that the presence of a lupus anticoagulant is more predictive of thrombosis than high titers of anti-cardiolipin or anti-beta-2-glycoprotein.

Testing for lupus anticoagulant in the presence of anticoagulant therapy (including warfarin, direct thrombin inhibitors & direct factor 10a inhibitors, and supratherapeutic heparin) is not recommended due to possible interference with test results. The presence of factor deficiencies or a factor specific inhibitor may also interfere with this assay.
24 B2GPG Beta-2-glycoprotein Antibody, IgG In the context of diagnosing anti-phospholipid syndrome, anti-beta-2-glycoprotein IgG and IgM should be performed in conjunction with other laboratory tests (all pertinent labs are included in the anti-phopholipid antibody panel) in patients who meet clinical criteria as defined in the revised Sapporo criteria (J Thromb Haemost. 2006;4(2):295). The laboratory evaluation should take place at least 12 weeks after a qualifying clinical event and be repeated at least 12 weeks after the first round of tests. We do not recommend testing for anti-phospholipid syndrome at the time of an acute event. The presence of clinical criteria and at least one positive laboratory criteria (with repeat positivity at least 12 weeks apart) establishes a diagnosis of anti-phospholipid syndrome.

Studies have shown that the association of thrombosis is strenghtened with high titers and IgG subtype of anti-beta-2-glycoprotein. Studies have shown that IgG or IgM anti-beta-2-glycoprotein demonstrate weaker association with thrombosis than a positive lupus anticoagulant test.

Anti-beta-2-glycoprotein is more specific than anti-cardiolipin.

 

Clinical Algorithm:

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