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.
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.
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.
Treatment of recurrent thrombosis despite anticoagulation
Management of noncriteria manifestations
Limited role of alternative therapies
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.
Immunomodulatory agents
Treatment of catastrophic disease
Optimal treatment has not been clearly defined in this condition. We are limited to data from small case report studies. These patients often display a fulminant course with rapid multiorgan system failure, so multiple interventions are often desperately tried in hopes that the patient might respond to something and survive.