Medical Care: Pregnant women with APS are considered high-risk obstetric patients, and medical care is instituted with this in mind. If a chromosomal abnormality is found, genetic counseling is recommended.
Intravenous immunoglobulin (IVIG): Infused immunoglobulins may modulate aCL antibodies levels by 3 mechanisms.
Antiidiotypic antibodies may be present in the IVIG preparation. These antiidiotypic antibodies may bind autoantibodies to form idiotype-antiidiotype dimers, resulting in neutralization of autoantibody effects.
Antiidiotype antibodies may bind and down-regulate B-cell receptors, resulting in a decrease in autoantibody production.
Antiidiotype antibodies might bind receptors of regulatory T cells, resulting in suppression of lymphokine production and decreased activation of autoantibody-producing B cells.
Landry-Guillain-BarrΓ©-Strohl syndrome (LGBSS) of acute inflammatory demyelinating polyradiculoneuropathy
Patients usually present with progressive bilateral and symmetrical muscle weakness accompanied by mild sensory symptoms, including paresthesia, numbness, and tingling. The disease can progress to involve the respiratory muscles, resulting in respiratory failure. Two thirds of the patients have a history of viral-like infections 1-3 weeks prior to the onset of symptoms.
Recently, CMV infection has been incriminated as a potential etiologic agent in some pregnant patients presenting with LGBSS.
Acute inflammatory demyelinating polyradiculoneuropathy is a rare disease with an incidence of approximately 1-1.5 cases per 100,000 LGBSS cases per year. LGBSS is exceedingly rare in pregnancy.
Obstetric care
Patients should be counseled in all cases regarding symptoms of thrombosis and thromboembolism and should be educated regarding and examined frequently for the signs or symptoms of thrombosis or thromboembolism, severe PIH, or decreased fetal movement.
In patients with poor obstetric histories, evidence of PIH, or evidence of fetal growth restriction, ultrasonography is recommended every 3-4 weeks starting at 18-20 weeks of gestation.
Human chorionic gonadotropin (hCG) values may suggest early prognosis of the pregnancy outcome in the first trimester. If hCG levels are increasing normally (ie, doubling every 2 d) in the first month of pregnancy, a successful outcome is predicted in 80-90% of cases. However, when the increases are abnormal (ie, slower), a poor outcome is predicted in 70-80% of cases.
In patients with uncomplicated APS, ultrasonography is recommended at 30-32 weeks of gestation (ie, to evaluate uteroplacental sufficiency).
Low molecular weight heparin (LMWH) may be used in APS and pregnancy (replacement of unfractionated sodium heparin).
Importantly, counsel the patient regarding potential adverse effects of heparin. Heparin-induced osteoporosis occurs in 1-2% of cases.
Drugs such as chloroquine and cytotoxic agents are not recommended during pregnancy, and patients should stop taking these drugs several months prior to becoming pregnant.
Warfarin may be substituted for heparin during the postpartum period to limit further risk of heparin-induced osteoporosis and bone fracture.
Splenectomy during the early second trimester or at the time of cesarean delivery may be considered in patients refractory to glucocorticoid therapy.
Nonobstetric care
Immunosuppressive agents are recommended for patients with SLE with secondary APS.
Thromboprophylaxis is recommended.
Patients should be evaluated for renal disease, (glomerulonephritis, N-stage disease), anemia, and thrombocytopenia.
PROPOSED MANAGEMENT FOR WOMEN WITH APL ANTIBODIES :
Note the following:
The medications shown should not be used in the presence of contraindications.
Close obstetric monitoring of the mother and fetus is necessary in all cases.
The patient should be counseled in all cases regarding symptoms of thrombosis and thromboembolism.
Surgical Care:
Cardiac valvular surgery is recommended in patients with severe aortic regurgitation as a result of APS.
Splenectomy is recommended in patients with the chronic form of idiopathic thrombocytopenic purpura and is associated with remission in approximately 75% cases.
Thromboprophylaxis is recommended for any abdominal or orthopedic surgery.
Manage thrombotic or hemorrhagic complications. Be aware of associated thrombocytopenia, and employ laboratory methods of perioperative anticoagulation monitoring in the setting of prolonged clotting times.
DRUG TREATMENT : In women with well-recognized obstetric APS, anticoagulant prophylaxis is recommended during pregnancy and the postpartum period. Pregnant women with APS are considered at risk of thrombosis and pregnancy loss. Data suggest low-dose aspirin and heparin (either unfractionated heparin or LMWH) are the treatments of choice for prevention of pregnancy loss in pregnant women with APS and previous pregnancy losses. Pregnant women with APS and a history of thrombosis but no pregnancy loss only require treatment with heparin. Treatment is optional for patients with no history of pregnancy loss or thrombosis.
Drug Category: Heparin compounds -- Unfractionated IV heparin and fractionated SC LMWH are the 2 choices for initial anticoagulation therapy. Warfarin therapy may be initiated in the postpartum stage.
These are used in the treatment or prophylaxis of clinically evident intravascular thrombosis. Special precaution should be exercised in obstetrical emergencies or massive liver failure.
LMWHs may also be used. Similar to unfractionated heparin, LMWHs are a class of anticoagulants termed glycosaminoglycans. LMWHs are derived from unfractionated heparin but have smaller, more standard average masses than heterogeneous unfractionated heparin.
Unlike standard heparin, LMWHs have higher specificity for factor Xa and have fewer effects on platelet activity. As a result, LMWH may cause bleeding less often, while still retaining anticoagulant effects. LMWHs may be associated with less risk of heparin-induced osteoporosis.
- HEPARIN
- ENOXAPARIN
2. ANTIPLATELET AGENTS : Randomized controlled trials demonstrate improved fetal survival when pregnant women with APS and prior pregnancy losses are treated with low-dose aspirin plus heparin compared with low-dose aspirin alone.
- ASPIRIN