Antiphospholipid Antibody Syndrome and Pregnancy Treatment & Management

  • Author: Teresa G Berg, MD, FACOG; Chief Editor: Carl V Smith, MD   more...
 
Updated: Jan 14, 2011
 

Medical Care

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

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 preeclampsia, or decreased fetal movement.

In patients with poor obstetric histories, evidence of preeclampsia, or evidence of fetal growth restriction, ultrasonography is recommended every 3-4 weeks starting at 18-20 weeks’ gestation.

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 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. Initiation of heparin in the face of a failing pregnancy should be undertaken with caution due to bleeding risks.

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.

Anticoagulation with heparin is recommended in APS and pregnancy. 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.

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 with thrombocytopenia refractory to glucocorticoid therapy.

Bone density studies should be considered in patients receiving anticoagulation 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.

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-Barré-Strohl syndrome (LGBSS) of acute inflammatory demyelinating polyradiculoneuropathy can be seen in patients with APS and lupus.

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.

Nonobstetric care

Immunosuppressive agents are recommended for patients with SLE with secondary APS.

Thromboprophylaxis is recommended.

Patients should be evaluated for renal disease, (glomerulonephritis, end-stage renal disease), anemia, and thrombocytopenia.

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

FeatureManagement
PregnantNonpregnant
APS with prior fetal death or recurrent pregnancy lossHeparin in prophylactic doses (15,000-20,000 U of unfractionated heparin or equivalent per d) 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 strokeHeparin 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 thrombosisNo 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
LGBSSHigh-dose IVIG at 400-1500 mg/kg/d for several daysIVIG at 400-1500 mg/kg/d for several days
aPL Antibodies Without APS
LAC or medium-to-high level of aCL IgGNo treatmentNo treatment
Low levels of aCL IgG, only aCL IgM, or only aCL IgA without LA, aPL, or aCLNo treatmentNo treatment

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.
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Surgical Care

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.
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Consultations

  • 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 one or more prior thrombotic event, lifelong anticoagulation with warfarin may be advisable to avoid recurrent thrombosis. An assessment by a Rheumatologist or Hematologist may also be helpful.
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Contributor Information and Disclosures
Author

Teresa G Berg, MD, FACOG  Associate Professor, Program Director, Director of the Perinatal Diagnostic Center, Department of Obstetrics and Gynecology, University of Nebraska Medical Center

Teresa G Berg, MD, FACOG is a member of the following medical societies: American Institute of Ultrasound in Medicine, Association of Professors of Gynecology and Obstetrics, Central Association of Obstetricians and Gynecologists, and Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Bruce A Meyer, MD, MBA  Executive Vice President for Health System Affairs, Chief Clinical Officer, Interim CEO, University Hospitals; Professor, Department of Obstetrics and Gynecology, University of Texas Southwestern Medical School

Bruce A Meyer, MD, MBA is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Physician Executives, American Institute of Ultrasound in Medicine, Association of Professors of Gynecology and Obstetrics, Massachusetts Medical Society, Medical Group Management Association, and Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Frederick B Gaupp, MD  Consulting Staff, Department of Family Practice, Hancock Medical Center

Frederick B Gaupp, MD is a member of the following medical societies: American Academy of Family Physicians

Disclosure: Nothing to disclose.

Chief Editor

Carl V Smith, MD  The Distinguished Chris J and Marie A Olson Chair of Obstetrics and Gynecology, Professor, Department of Obstetrics and Gynecology, University of Nebraska Medical Center

Carl V Smith, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, Association of Professors of Gynecology and Obstetrics, Central Association of Obstetricians and Gynecologists, Council of University Chairs of Obstetrics and Gynecology, Nebraska Medical Association, and Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

References

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Table. Proposed Management for Women With aPL Antibodies
FeatureManagement
PregnantNonpregnant
APS with prior fetal death or recurrent pregnancy lossHeparin in prophylactic doses (15,000-20,000 U of unfractionated heparin or equivalent per d) 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 strokeHeparin 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 thrombosisNo 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
LGBSSHigh-dose IVIG at 400-1500 mg/kg/d for several daysIVIG at 400-1500 mg/kg/d for several days
aPL Antibodies Without APS
LAC or medium-to-high level of aCL IgGNo treatmentNo treatment
Low levels of aCL IgG, only aCL IgM, or only aCL IgA without LA, aPL, or aCLNo treatmentNo treatment
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