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.
Platelet inhibition
Aspirin is frequently added to a regimen of chronic anticoagulation, particularly when patients experience recurrent thrombosis despite therapeutic aticoagulation. However data demonstrating additive benefit are lacking.
Immunosuppression
It is not clear that immunosuppression is beneficial, particularly in patients who do not have an underlying autoimmune process. Nevertheless, immunosuppression is often tried in patients who have failed usual anticoagulation. Steroids, for example prednisone 1 mg/kg (or equivalent), has been used with moderate success. Pulse solumedrol IV 1 g/d for 3 days is an alternative regimen. Cyclophosphamide, either oral or pulse IV, has demonstrated modest utility.
Other, more desperate interventions include intravenous immunoglobulin and plasmapheresis. The latter has been shown via case reports to have efficacy in patients who have failed other interventions.
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.