Antiphospholipid Syndrome and Pregnancy Treatment & Management

Pregnant women with APS are considered high-risk obstetric patients, and medical care is instituted with this in mind.

In patients receiving or recently treated with corticosteroid therapy, administer supplementation to cover the labor or cesarean delivery.

Pregnancy in itself is not harmful to the mother or the baby unless added work related to the newborn, as well as emotional stress in the family, proves to be too much for a particular patient. Therapeutic abortions are generally not indicated in pregnant women with autoimmune disease.

Epidural anesthetic is not recommended if the mother has a marked drop in the maternal platelet count. The use of forceps or the vacuum extractor should be individualized.

No evidence indicates adverse effects related to breastfeeding, although breastfeeding is not recommended if high doses of cytotoxic or immunosuppressive agents are required.

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 preeclampsia, or decreased fetal movement.

Ultrasonography is recommended every 3-4 weeks starting at 18-20 weeks’ gestation, in patients with a poor obstetric history, evidence of preeclampsia, or evidence of fetal growth restriction.

Human chorionic gonadotropin (hCG) values in the first trimester can be followed to evaluate the viability of the pregnancy. If hCG levels are increasing normally (ie, doubling every 2 days) 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’ gestation to assess fetal growth. Lagging fetal growth may reflect uteroplacental insufficiency in patients with APS.

Drugs such as chloroquine and cytotoxic agents are not recommended during pregnancy; patients should stop taking these drugs several months prior to becoming pregnant.

Splenectomy during the early second trimester or at the time of cesarean delivery may be considered in patients with thrombocytopenia refractory to glucocorticoid therapy.

Anticoagulation therapy

Anticoagulation with heparin is recommended in APS and pregnancy with a history of a thromboembolic event. Low-molecular-weight heparin (LMWH) may be used in these patients.

Importantly, counsel the patient regarding potential adverse effects of heparin. Heparin-induced osteoporosis occurs in 1-2% of cases. Initiation of heparin in the face of a failing pregnancy should be undertaken with caution due to bleeding risks.

Bone density studies should be considered in patients receiving anticoagulation therapy with heparin or LMWH due to the risks of osteopenia. This may be most important in women who have been treated in a previous pregnancy or are planning pregnancy.

Warfarin may be substituted for heparin during the postpartum period to limit further risk of heparin-induced osteoporosis and bone fracture.

In women without a history of a thromboembolic event, optimal therapy is not as clear. Anticoagulation may decrease recurrent pregnancy loss in this group of women. Low-dose aspirin combined with prophylactic doses of heparin or LMWH appears to be superior to aspirin therapy alone or maternal steroids.

Lefkou et al studied 21 pregnant women with APS who developed preeclampsia and/or intrauterine growth restriction during treatment with low-dose aspirin plus low-molecular weight heparin (LDA+LMWH ) and found increased placental blood flow and improvement in preeclampsia features in the patients who received both pravastatin and LDA+LMWH. ^[11]

Intravenous immunoglobulin

Infused immunoglobulins may modulate aCL antibodies levels by the following 3 mechanisms:

• Antiidiotypic antibodies may be present in the intravenous immunoglobulin (IVIG) preparation; these antiidiotypic antibodies may bind autoantibodies to form idiotype-antiidiotype dimers, resulting in neutralization of autoantibody effects.

• Antiidiotype antibodies may bind and downregulate 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

Immunosuppressive agents are recommended for patients with SLE with secondary APS. Thromboprophylaxis is also recommended. In addition, patients should be evaluated for renal disease, (glomerulonephritis, end-stage renal disease), anemia, and thrombocytopenia. (See the Table below.)

Table. Proposed Management for Women With aPL Antibodies (Open Table in a new window)

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

Patients with APS, especially secondary APS, may require surgical interventions for long-standing complications of their autoimmune disorder.

Cardiac valvular surgery is recommended in patients with severe aortic regurgitation due to the noninfectious vegetations that are seen 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 use laboratory methods of perioperative anticoagulation monitoring in the setting of prolonged clotting times.

The patient should be informed about potential maternal and obstetric problems, including fetal loss, thrombosis or stroke, PIH, fetal growth restriction, and preterm delivery. Consultation with specialists in Maternal-Fetal Medicine and Rheumatology should be considered.

In women with APS and 1 or more prior thrombotic events, lifelong anticoagulation with warfarin may be advisable to avoid recurrent thrombosis. An assessment by a rheumatologist or hematologist may also be helpful.