Antiphospholipid syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]

Overview

The most important therapy for symptomatic antiphospholipid syndrome is platelet inhibition with or without anticoagulation. Platelet inhibition is often achieved with aspirin, while warfarin and heparin are the mainstays of anticoagulation. Typically, there is no indication for primary prophylaxis. Immunosuppression, the use of intravenous immunoglobulin, and plasmapheresis have also been used with modest success in patients with catastrophic antiphospholipid syndrome (APS).

Medical Therapy

General principles and choice of anticoagulation

The mainstay of treatment in antiphospholipid syndrome(APS) is anticoagulation. The choice of anticoagulant is heparin, which is given in overlap with warfarin. In cases where warfarin is contraindicated such as pregnancy, low molecular weight heparin (LMWH) is used.

1.Treatment of acute thromosis in APS

• The choice of treatment for acute thrombosis in APS is low molecular weight heparin (LMWH).
• It is overlapped with warfarin for a minimum of 4-5 days.
• It is continued as long as the International normalized ratio (INR) is in the therapeutic range that is 2-3.

4.Anticoagulation in pregnancy

• During pregnancy, low molecular weight heparin and low-dose aspirin are used to avoid warfarin's teratogenicity.
• The therapy is initiated at the beginning of pregnancy and continued until the time of delivery.
• Women with recurrent miscarriage are often advised to take low dose aspirin and to start low molecular weight heparin treatment after missing a menstrual cycle.
• For women with previous history of clots, higher dose of low molecular weight heparin is used.

Treatment of refractory cases in pregnancy

• Intravenous immunoglobulin(IgG) and corticosteroids are used for patients with refractory cases in pregnancy.

5.Limited role of alternative therapies:

Following alternative therapies can be used for the treatment of APS:

Direct oral anticoagulants:

The rationale behind using direct oral anticoagulants such as dabigatran, apixaban or rivaroxaban is as follows:

• They dont require laboratory monitoring of PT/aPTT.
• They have lower risk of bleeding.
• They are useful for patients who cannot tolerate warfarin.

Immunomodulatory agents:

Immunomodulatory agents are proposed for the use of antiphospholipid syndrome as it an autoimmune disease. The following drugs are preferred:

• Rituximab
• Hydroxyychloroquine

6.Treatment of catastrophic antiphospholipid syndrome:

A small subset of patients develop catastrophic disease which is managed as follows:

Pearls of management:

Early diagnosis and timely management with adressing the thrombotic events and suppressing the cytokine cascade is essential for the treatment.

Approach to treatment:

The steps of managing catastrophic APS are as follows:

Antibiotics:

• Identify the underlying infection and administer appropriate antibiotics accordingly.

Anticoagualtion:

• Anticoagulate with heparin in the acute setting.
• In hemodynamically stable patients and no evidence of bleeding, oral anticoagulant such as warfarin can be used.

Systemic glucocorticoids:

• Preferred regimen (1): Methylprednisone 0.5-1g IV q12h for 3 days

This is followed by oral therapy with 1mg/kg of prednisone per day.