Antiphospholipid Syndrome Treatment & Management

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Treatment

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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