Diagnostic Considerations
Careful evaluation is necessary following a spontaneous abortion occurring prior to 10 weeks’ gestation before it can be considered unexplained.
With regard to thrombocytopenia, other possible causes of the disorder, such as HIV infection, and various conditions, such as drug -induced thrombocytopenia, thrombotic thrombocytopenia, gestational thrombocytopenia, and preeclampsia, should be considered. Other autoimmune diseases should also be ruled out in patients with thrombocytopenia.
The following are findings that can be secondary to autoimmune disorders other than APS and that require an appropriate evaluation:
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Arthritis - Patients may have nonerosive arthritis involving 2 or more peripheral joints
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Serositis - This may be (1) pleuritis or pleural effusion or (2) pericarditis or pericardial effusion
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Renal disorder - Proteinuria of 0.5 g/day or the presence of cellular casts
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Neurologic disorder - Criteria include seizures in the absence of other causes or psychosis in the absence of other causes.
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Hematologic disorder - Features, without an otherwise explainable cause, include (1) hemolytic anemia with reticulocytosis, (2) leukopenia of less than 4000 cells/µL on at least 2 occasions, (3) lymphopenia of less than 1500 cells/µL, or (4) thrombocytopenia of less than 100,000 cells/µL
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Immunologic disorder
Systemic lupus erythematosus
Many manifestations of SLE can be seen in women with APS. A complete evaluation to exclude SLE as a primary disorder is appropriate if symptoms are present.
The clinical manifestations of SLE include the following:
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Skin lesions - 84-71%
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Arthritis - 63-95%
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Nephritis - 46-77%
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Raynaud phenomenon - 10-58%
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Neuropsychiatric features - 0-59%
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Lymphadenopathy - 0-58%
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Pleurisy - 37-56%
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Mucous membrane ulceration - 7-54%
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Pericarditis - 29-45%
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Splenomegaly - 9-18%
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Aseptic necrosis - 0-10%
Clinical evidence of glomerulonephritis is found in more than 50% of cases. However, if biopsies are performed on all patients, the incidence of some nephritis may be as high as 90%. SLE is associated with encephalopathy and seizures, to a lesser degree with ischemic stroke, and, rarely, with subarachnoid hemorrhage.
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 |
