Antiphospholipid Syndrome Treatment & Management

  • Author: Elise Belilos, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jan 19, 2012
 

Medical Care

Patients with antiphospholipid syndrome (APS) may be evaluated in an outpatient setting. Inpatient evaluation is required if the patient presents with a significant clinical event. Patients with CAPS require intense observation and treatment, often in an intensive care unit.

In general, treatment regimens for APS must be individualized according to the patient's current clinical status and history of thrombotic events. Asymptomatic individuals in whom blood test findings are positive do not require specific treatment.

  • Prophylactic therapy
    • Eliminate other risk factors, such as oral contraceptives, smoking, hypertension, or hyperlipidemia.
    • Low-dose aspirin is used widely in this setting; however, the effectiveness of low-dose aspirin as primary prevention for APS remains unproven. Clopidogrel has anecdotally been reported to be helpful in persons with APS and may be useful in patients allergic to aspirin.
    • In patients with SLE, consider hydroxychloroquine, which may have intrinsic antithrombotic properties.
    • Consider the use of statins, especially in patients with hyperlipidemia.
  • Thrombosis
    • Perform full anticoagulation with intravenous or subcutaneous heparin followed by warfarin therapy.
    • Based on the most recent evidence, a reasonable target for the international normalized ratio (INR) is 2.0-3.0 for venous thrombosis and 3.0 for arterial thrombosis. Patients with recurrent thrombotic events, while well maintained on the above regimens, may require an INR of 3.0-4.0. For severe or refractory cases, a combination of warfarin and aspirin may be used. Treatment for significant thrombotic events in patients with APS is generally lifelong.
  • Obstetric considerations
    • Guidelines from the American College of Obstetricians and Gynecologists (based primarily on consensus and expert opinion [level C]) regarding prenatal and postpartum care for women with APS recommend prophylaxis for those with no history of thrombosis and full anticoagulation for those with a history of thrombosis.[10, 11]
    • Patients with pregnancy loss receive prophylactic subcutaneous heparin (preferably low–molecular-weight heparin [LMWH]) and low-dose aspirin. Therapy is withheld at the time of delivery and is restarted after delivery, continuing for 6-12 weeks postpartum. Warfarin (Coumadin) is contraindicated in pregnancy.
    • Patients with a history of thrombosis receive therapeutic doses of heparin during pregnancy; long-term anticoagulation is then continued postpartum.
    • Corticosteroids have not been proven effective for persons with primary APS, and they have been shown to increase maternal morbidity and fetal prematurity rates.
    • Breastfeeding women may use heparin and warfarin.
  • CAPS
    • These patients are generally very ill, often with active SLE.
    • Treatment with intensive anticoagulation, plasma exchange, and corticosteroids appears beneficial, but no controlled trials have been performed. Intravenous immunoglobulin may be of some benefit and cyclophosphamide may be considered in selected cases, especially in SLE-associated CAPS.
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Surgical Care

Recurrent DVT may necessitate placement of an inferior vena cava filter.

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Consultations

  • Rheumatologist
  • Hematologist
  • Neurologist, cardiologist, pulmonologist, hepatologist, ophthalmologist (depending on clinical presentation)
  • Obstetrician with experience in high-risk pregnancies
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Diet

  • If warfarin therapy is instituted, instruct the patient to avoid excessive consumption of foods that contain vitamin K.[12]
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Activity

  • No specific limitations on activity are necessary.
  • Individualize the activity according to the clinical setting.
  • Instruct the patient to avoid sports with excessive contact if taking warfarin.
  • Limit activity in patients with acute DVT.
  • Instruct the patient to avoid prolonged immobilization.
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Contributor Information and Disclosures
Author

Elise Belilos, MD  Section Head of Rheumatology, Division of Rheumatology, Allergy and Immunology, Winthrop-University Hospital; Assistant Professor of Clinical Medicine, Department of Internal Medicine, State University of New York at Stony Brook

Elise Belilos, MD is a member of the following medical societies: American College of Rheumatology and Arthritis Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Steven Carsons, MD  Chief, Division of Rheumatology, Allergy, and Immunology, Professor of Medicine, Department of Internal Medicine, Winthrop University Hospital, State University of New York at Stony Brook

Steven Carsons, MD is a member of the following medical societies: American College of Rheumatology, New York Academy of Sciences, and Society for Experimental Biology and Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Carlos J Lozada, MD  Director of Rheumatology Fellowship Program, Professor, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Pfizer Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

The authors gratefully acknowledge the contributions of Amiel Tokayer, MD.

References

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