Q1: "My hemo just ran five new tests on me, looking for another cause of clotting. It looks as if the beta-2-glycoprotein test is an indicator of APS (= antiphospholipid antibody syndrome). My IgG and IgA are both normal, but my IgM is 34 (should be

Wien Klin Wochenschr. 2000 Aug 25;112(15-16):707-10. Antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.

Ann Emerg Med. 1992 Feb;21(2):207-11. We present the case of a 26-year-old man with an exacerbation of apparent chronic asthma with chronic peripheral vascular disease due to recurrent venous thrombosis. Localized livedo reticularis, new cutaneous infarctions, severe venous insufficiency, thrombocytopenia, renal failure, and cerebral supratentorial dysfunction were noted. During hospital admission, antibodies to phospholipids in high titer were present by three different testing methods. Renal biopsy demonstrated significant renal vasculature abnormalities characteristic of hemolytic endovasculopathy, and magnetic resonance imaging showed multiple cerebral infarctions. This case exemplifies the spectrum of presentations and management of the primary antiphospholipid antibody syndrome. The clue to its presence in this patient was the livedo reticularis rash, a cutaneous marker for this syndrome that was evident in the emergency department.

Example: Q1: "Could you please explain more about the effect of lupus anticoagulants on the INR tests? My hematologist has not heard of the difficulties of the two interacting with each other. Are there any references you could provide so I can give them to my doc? I have not been able to maintain a consistent INR level; it is either at 1.8 or 3.8, very rarely does it come in between 2-3."

Finding May be First Step in Preventing Premature Stroke, Heart Attack and Miscarriage

The British Journal of Rheumatology, Vol 37, 1229-1232, Copyright © 1998 by British Society for Rheumatology

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Antiphospholipid syndrome (APS) is familial in 10% of cases.

Posted on: Tuesday, 14 February 2006, 03:03 CST

Blood, 1 March 2003, Vol. 101, No. 5, pp. 1827-1832

N. Engl. J. Med. 332:993-997 April 1995. Conclusions The risk of recurrent thrombosis in patients with the antiphospholipid-antibody syndrome is high. Long-term anticoagulation therapy in which the international normalized ratio is maintained at or above 3 is advisable in these patients.

Last Updated: 2/15/2004

Hematology 2003 © 2003 The American Society of Hematology

Rev Med Interne. 2002 Apr;23(4):357-63. PURPOSE: The association of antiphosphatidylethanolamine antibodies (aPE) as the only antiphospholipid antibody with antiphospholipid syndrome (APS) is discussed. The aPE was described as the sole antibody in many cases suggesting APS. aPE was not included in the Sapporo criteria for the classification of APS. METHODS: We investigated the clinical features of 20 patients with aPE only; 17 patients had symptoms potentially related to APS (group 1) and three had other manifestations (group 2). RESULTS: There were 15 women and five men, mean age was 35 +/- 12 years at the beginning. In group 1 (n = 17), ten patients presented arterial thrombosis, nine venous thrombosis (five had both), and six microvascular thrombosis (livedo reticularis, lacunar pathology). The aPE positivity was persistent in 13 patients. A subgroup of four patients (three women) presented arteriosclerosis with peripheral arteriopathy which started before 45 years of age. They had another atherosclerosis risk factor associated with aPE persistence. In group 2 (n = 3), there was no thrombotic event, one demyelinating pathology, one microvascular pathology, and one arterial dysplasia. The aPE positivity was never confirmed. Finally, 13 patients presented an APS with aPE only, confirmed at least 8 weeks later. CONCLUSIONS: Our study points out that testing for aPE would be of interest for patients when symptoms were potentially related to APS, particularly when other antiphospholipid antibodies were negative. This description questions the enlargement of the APS biological criteria defined in Sapporo. The role of aPE in atherosclerosis is considered.

Arch Pathol Lab Med—Vol 131, June 2007. Conclusions: Although there are published criteria for confirming the presence of a lupus anticoagulant, there is no consensus on assay methods for lupus anticoagulant.

Acquired thrombophilia, antiphospholipid antibodies (APAs) and the antiphospholipid antibody syndrome (APS) are autoimmune conditions characterized by the presence of certain clinical features and levels of circulating APAs.

Journal of Autoimmunity. Volume 30, Issues 1-2, February-March 2008, Pages 99-103. Autoimmunity: From the Mosaic to the Kaleidoscope. The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by an adaptive immune response against self-PL-binding proteins ending in the production of specific autoantibodies. Antiphospholipid antibodies (aPL; and in particular anti-β2 glycoprotein I antibodies) are formal diagnostic markers and pathogenic antibodies. Although APS may be considered as an autoantibody-mediated disease, there is now evidence that aPL are necessary but not sufficient to trigger some of the clinical manifestations of the syndrome. For example, additional factors, such as mediators of the innate immunity are now recognized to play a key role as second hits able to induce the thrombotic events in the presence of the autoantibodies. The APS scenario is also supplemented by the influence of genetically determined factors. Finally, environmental agents – in particular infectious ones – were reported to act as triggers for the production of autoantibodies cross-reacting with PL-binding proteins as well as inflammatory stimuli that potentiate the aPL thrombogenic effect. Altogether these findings do support the concept of a mosaic of factors that participate to the pathogenesis of the syndrome at different levels.

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Circulation. 1997;96:4380-4384. Conclusions Hydroxychloroquine significantly diminished both thrombus size and total time of thrombus formation in mice previously injected with IgG-APS.

QJM, Vol 91, Issue 9 619-626, Copyright © 1998 by Oxford University Press

Clin Exp Rheumatol. 1989 Sep-Oct;7 Suppl 3:S85-9. If antibodies directed against the phospholipid itself play any part in the pathogenesis of the thrombotic events, the very ubiquitous nature of the antigen dictates that no single pathogenetic mechanism can be implicated. The majority of in vivo studies recently performed have failed to show that antiphospholipid (aPL) antibodies bind cultured endothelial cells, or that they affect prostacyclin production by these cells. Previously postulated actions showing defective fibrinolysis and impairment of coagulation inhibitors (protein C, thrombomodulin and antithrombin III) have yet to be substantiated. Studies have thus far failed to demonstrate any significant binding of aPL antibodies to intact platelets, although binding to disrupted platelets has been documented.

Blood, 1 June 2006, Vol. 107, No. 11, pp. 4195-4196.

First Release July 1 2006; J Rheumatol 2006;33:1559–62. Conclusion. Interpretation of a positive determination of APL is difficult in the elderly; persistent LAC may be the most valuable biological marker of APS in the elderly.

JAMA 2004;291:576-584.

Rheumatology 2000; 39: 421-426 © 2000 British Society for Rheumatology