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Antiphospholipid Syndrome Treatment & Management

  • Author: Suneel Movva, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Jul 01, 2016
 

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. Prophylaxis is needed during surgery or hospitalization, as well as management of any associated autoimmune disease.

Low-dose aspirin is used widely in this setting; however, the effectiveness of low-dose aspirin as primary prevention for APS remains unproven[8] . 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.

Treatment of 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 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.

No data exist regarding new oral anticoagulants (ie, direct thrombin inhibitors and factor Xa inhibitors) in APS patients. Currently, these agents can be considered in patients who are warfarin intolerant/allergic or have poor anticoagulant control.[8, 16] Two studies of the factor Xa inhibitor rivaroxaban are currently in progress: Rivaroxaban for Antiphospholipid Syndrome (RAPS) and Rivaroxaban in Antiphospholipid Syndrome (TRAPS).

Rituximab can be considered for recurrent thrombosis despite adequate anticoagulation. A nonrandomized prospective study showed rituximab to be effective for noncriteria aPL manifestations (ie, thrombocytopenia and skin ulcers).[5]

Obstetric considerations

Guidelines from the American College of Obstetricians and Gynecologists (based primarily on consensus and expert opinion [level C]) recommend that women with APS who have a history of thrombosis in previous pregnancies receive prophylactic anticoagulation during pregnancy and for 6 weeks postpartum. For women with APS who have no history of thrombosis, the guidelines suggest that clinical surveillance or prophylactic heparin use antepartum, along with 6 weeks of postpartum anticoagulation, may be warranted.[17]

Prophylaxis during pregnancy is provided with 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, or long-term in patients with a history of thrombosis.

Warfarin (Coumadin) is contraindicated in pregnancy. Breastfeeding women may use heparin and warfarin.

Corticosteroids have not been proven effective for persons with primary APS, and they have been shown to increase maternal morbidity and fetal prematurity rates.

Catastrophic APS

Patients with catastrophic APS (CAPS) are generally very ill, often with active SLE. Treatment (which is not based on controlled trials) consists of attention to associated disorders (eg, infection, SLE) and intensive anticoagulation, with consideration of the following[18] :

  • Plasma exchange
  • Cyclophosphamide, in patients with SLE
  • In refractory or relapsing cases, new therapies, such as rituximab and possibly eculizumab

 

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Surgical Care

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

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Consultations

Consultations may include the following:

  • 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.[19]

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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

Suneel Movva, MD Fellow in Rheumatology, Division of Rheumatology, Allergy and Immunology, Winthrop University Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Steven Carsons, MD Chief, Division of Rheumatology, Allergy and Immunology, Winthrop University Hospital; Professor of Medicine, Stony Brook University School of Medicine

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

Disclosure: Nothing to disclose.

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, Stony Brook University School of Medicine

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Carlos J Lozada, MD Director of Rheumatology Fellowship Training Program, Professor of Clinical Medicine, 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, American College of Rheumatology

Disclosure: Received honoraria from Pfizer for consulting; Received grant/research funds from AbbVie for other; Received honoraria from Heel for consulting.

Acknowledgements

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

References
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