Doctor Hughes Book on a website

US Pharm. 2008;33(1):HS-23-HS-30. Conclusion: APS is an autoimmune disorder that presents unique challenges in diagnosis and treatment. The revised diagnosis can be simply stated as the presence of one clinical and one laboratory criteria. In practice, the heterogeneous presentation of both clinical and laboratory findings complicates diagnosis. This disorder should always be included in differential diagnosis of persons who have coagulation defects, evidence of vascular thrombosis, and/or history of recurrent miscarriages or fetal losses. Positive laboratory testing should always be confirmed at least 12 weeks apart to verify persistence. The heterogeneous presentation of APS also makes treatment challenging, and, in particular, research clarifying optimal therapy remains lacking. Currently, the mainstay of treatment is anticoagulation in those persons who develop an acute thrombotic event. Although indefinite duration of anticoagulation therapy is recommended, the decision to administer long-term anticoagulation certainly requires judicious clinical evaluation and risk assessment, given the potential hemorrhagic complications of anticoagulation therapy.

Indian Pediatrics 2001; 38: 1413-1416 Promotes an INR greater than 3.

An. Bras. Dermatol. vol.80 no.3 Rio de Janeiro May/June 2005. Antiphospholipid syndrome is an acquired multisystem disorder characterized by recurrent thromboses in the arterial system, venous system, or both. Antiphospholipid syndrome is classified into 2 groups: primary and secondary. Secondary antiphospholipid syndrome is often associated with systemic lupus erythematosus and less frequently with infections, drugs and other diseases. Serologic markers are antiphospholipid antibodies, lupus anticoagulant and anticardiolipin. The primary diagnostic criteria include arterial thrombosis or venous thrombosis and recurrent fetal loss. About 41% of patients with lupus anticoagulant have skin lesions as the first sign of antiphospholipid syndrome. Cutaneous manifestations include livedo reticularis, cutaneous ulceration and livedo vasculitis. The mainstays of prophylaxis and treatment of thrombosis are anticoagulant and antiplatelet agents.

Medicine (Baltimore). 1989 Nov;68(6):366-74.

APSSA is a foundation formed to promote much needed awareness of the Antiphospholipid Syndrome (APS) in South Africa.

The antiphospholipid syndrome (APS) is characterized by venous and/or arterial thrombosis, recurrent pregnancy loss and the presence of antiphospholipid antibodies. The antiphospholipid antibodies (anticardiolipin, anti-bêta2GPI antibodies, lupus anticoagulant) interacting with various coagulation proteins, platelets or endothelial cells may contribute to disease pathogenesis. Incidence remains unknown, however the reported prevalence of antiphospholipid antibodies in the general population is low (1-4.5%) and increases with age. The main clinical manifestations associated with APS are thromboses, pregnancy morbidity, thrombocytopenia, neurological symptoms, livedo reticularis, hemolytic anemia. The antiphospholipid antibodies have been detected in approximately 1/3 of the patients with systemic lupus erythematosus (SLE). High anticardiolipin antibodies titers, lupus anticoagulant and especially anti-bêta2GPI antibodies are important predictors of APS clinical manifestations in SLE patients. The management of thrombosis includes long-term, high-intensity warfarin therapy [International Normalized Ratio (INR superior or equal to 3)]. For pregnancy morbidity the recommended therapy is low-dose aspirin (80 mg/day) plus subcutaneous unfractionated heparin or low-molecular-weight heparin.

Medical Author: William C. Shiel Jr., MD, FACP, FACR Last Editorial Review: 9/18/2005

Reported by Ronald A. AshersonI MD,FACP,MD(Hon)FRCP,FCP(SA)FACR,and Ricard Cervera2 MD,PhD

© 2004. The Journal of Rheumatology Publishing Company Limited.

© 2001. The Journal of Rheumatology Publishing Company Limited.

This page is in Italian.

PDF File from the New England Journal of Medicine

Last updated 01.08.2005 Written by Dr MY Karim, lecturer in immunology, St Thomas' Hospital and Dr GRV Hughes, consultant physician and rheumatologist, St Thomas' Hospital

Authored by Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital at Houston; Associate Professor, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Baylor College of Medicine Adjust dose to maintain an INR in the range of 2.5-3.5.

Another article that supports an INR of 3.0 to 4.0 for APS patients.

CMAJ • June 24, 2003; 168 (13) © 2003 Canadian Medical Association or its licensors

by Dr. Ron Ascherson

Posted 01/24/1997 Antiphospholipid (aPL) syndrome, or APS,--a cluster of conditions that includes arterial or venous thromboses and thrombocytopenia, as well as recurrent fetal loss associated with elevation of aPL antibody--has been reported to occur 2-5 times more frequently in women than men. Strong familial associations lead to the suspicion that aPL positivity, estimated to be present in 2% of the population, is a heritable trait in some cases. Currently, 2 major categories of the illness are recognized--primary and secondary. Secondary APS may be associated with autoimmune disease, malignancy, infectious disease, or drug-induced states. Two assays, one for lupus anticoagulant antibodies and the other for anticardiolipin (aCL) antibodies, are recognized to be the gold standards for serologic diagnosis of the disease. Despite extensive attempts at international standardization of aCL test results, no consensus exists for a value beyond which the test is considered positive. Interestingly, a "dose-effect" relationship for aCL antibody titers has been noted--higher titers of the antibody correlate with increased numbers of thrombotic events. An experimental assay for antibody against beta 2-glycoprotein 1 (beta-2-GP1), a phospholipid-binding protein, may become the most important assay for aPL. Skin findings in APS include livedo reticularis, ulceration, gangrene, or purpura, and, when present, may be the key to diagnosis of this sometimes insidious syndrome. Anticoagulation, usually with warfarin, is the mainstay of therapy, although steroids, immunosuppressive agents, hydroxychloroquine sulfate, and plasmapheresis may all be beneficial adjunctive therapy.

Official Web-site of the "European Forum on Antiphospholipid Antibodies"

Virtual Medical Centre. Modified: 14/7/2006

The MayoFoundation for Medical Education and Research provides the following web link addressing antiphospholipid syndrome.