Antiphospholipid syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Treatment
The cornerstone of therapy for symptomatic antiphospholipid syndrome hinges on platelet inhibition with or without anticoagulation. Platelet inhibition is often achieved with aspirin, while warfarin and heparin are the mainstays of anticoagulation. Generally there is no indication for primary prophylaxis. Immunosuppression, the use of intravenous immunoglobulin, and plasmapheresis have also been used with modest success.
Anticoagulation
When anticoagulation with warfarin is pursued, some authors recommend a goal INR of 3.0-4.0.[1] However, the current standard of care targets a therapeutic INR of 2.0-3.0 following initial venous thromboembolism, and an INR >3.0 for an arterial event or venous thrombosis refractory to anticoagulation.[2] Khamashta et al in a study of 147 patients with usual antiphopholipid antibody syndrome showed a low rate of recurrent thrombosis in patients with INR >3, with a risk of 7.1% bleeding complications per patient year (a third of which were serious).
Anticoagulation in pregnancy
During pregnancy, low molecular weight heparin and low-dose aspirin are used to avoid warfarin's teratogenicity. Women with recurrent miscarriage are often advised to take aspirin and to start low molecular weight heparin treatment after missing a menstrual cycle.
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.
References
- ↑ Horton JD, Bushwick BM (1999). "Warfarin therapy: evolving strategies in anticoagulation". American family physician. 59 (3): 635–646. PMID 10029789.
- ↑ Ruiz-Irastorza G, Hunt BJ, Khamashta MA (2007). "A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies". Arthritis and Rheumatism. 57 (8): 1487–95. doi:10.1002/art.23109. PMID 18050167. Unknown parameter
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