History
The “international consensus statement for the diagnosis of antiphospholipid syndrome,” published in 1999 by Wilson et al, serves as a set of criteria similar to that for the diagnosis of other autoimmune disorders. The criteria were updated in 2006 to reflect new insights into APS. The diagnosis requires that the patient have at least 1 clinical and 1 laboratory criterion. [7, 8]
Clinical and laboratory criteria
Clinical criteria
The clinical criteria for APS include the following:
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One or more clinical episodes of arterial, venous, or small-vessel thrombosis, occurring within any tissue or organ
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One or more unexplained deaths of morphologically normal fetuses at or after 10 weeks’ gestation
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One or more premature births of morphologically normal fetuses at or before 34 weeks’ gestation because of eclampsia or severe preeclampsia or features consistent with placental insufficiency
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Three or more consecutive, unexplained spontaneous abortions before 10 weeks’ gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded
Laboratory criteria
Criteria for laboratory testing, which are consistent with current clinical management guidelines from the American Congress of Obstetricians and Gynecologists, include the following [9] :
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Anticardiolipin antibodies - Anticardiolipin IgG or IgM antibodies present at moderate or high levels (ie, >40 GPL or MPL or >99th percentile) in the blood on 2 or more occasions at least 12 weeks apart
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Lupus anticoagulant - LAC detected in the blood on 2 or more occasions at least 12 weeks apart, according to the guidelines of the International Society on Thrombosis and Hemostasis [8]
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Anti-beta2 -glycoprotein I antibodies IgG or IgM - In titers above the 99th percentile for normal as defined by the laboratory performing the test, on 2 or more occasions at least 12 weeks apart
Additional findings in APS
Findings in APS can also include the following:
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Unexplained transient ischemic attacks or amaurosis fugax [1]
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Positive result from the Coombs test
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Hemolytic anemia
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Chorea
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Chorea gravidarum
Physical Examination
The diagnosis of APS is based primarily on clinical history and laboratory data. Patients with secondary APS are more likely to have findings on physical examination, although some physical findings may be associated with primary APS. Thrombosis and stroke are possible residual neurologic findings in APS.
Cutaneous manifestations of APS can include the following:
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Digital cyanosis
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Livedo reticularis
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Digital gangrene
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Leg ulcers
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Discoid rash - Ie, a raised, erythematous patch with keratotic scaling and follicular plugging; older lesions may be atrophic
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Photosensitivity
Tables
Feature |
Management |
|
Pregnant |
Nonpregnant |
|
APS with prior fetal death or recurrent pregnancy loss |
Heparin in prophylactic doses (15,000-20,000 U of unfractionated heparin or equivalent per day) administered subcutaneously in divided doses with low-dose aspirin daily Calcium and vitamin D supplementation |
Optimal management uncertain; options include no treatment or daily treatment with low-dose aspirin |
APS with prior thrombosis or stroke |
Heparin to achieve full anticoagulation (does not cross the placenta) |
Warfarin administered daily in doses to maintain international normalized ratio of =3 |
APS without prior pregnancy loss or thrombosis |
No treatment or daily treatment with low-dose aspirin or daily treatment with prophylactic doses of heparin plus low-dose aspirin; optimal management uncertain |
No treatment or daily treatment with low-dose aspirin; optimal management uncertain |
LGBSS |
High-dose IVIG at 400-1500 mg/kg/day for several days |
IVIG at 400-1500 mg/kg/d for several days |
aPL Antibodies Without APS |
||
LAC or medium to high level of aCL IgG |
No treatment |
No treatment |
Low levels of aCL IgG, only aCL IgM, or only aCL IgA without LA, aPL, or aCL |
No treatment |
No treatment |
