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The objective of this study was to assess the interest of antiphosphatidylethanolamine antibodies (aPE) in unexplained thrombosis (UT) defined as thrombotic episode without any of the main autoimmune and hereditary thrombophilic defects. Results from 98 UT were compared to those of (I) 142 patients with thrombophilia: 67 antiphospholipid syndrome (APS) and 75 hereditary hemostatic defects (HHD); (II) 110 patients without thrombosis: 60 with systemic lupus erythematosus (SLE) and 50 with infectious diseases (ID). As compared to controls (100 blood donors), aPE prevalence was significantly higher in both autoimmune contexts (APS: 43%; SLE: 40%, p<0.0001) and among non-autoimmune pathologies, only in UT (18%, p = 0.001) conversely to HHD (8%) or ID (10%). aPE prevalence in UT was not statistically different from that found in Primary APS (32%, p = 0.076) but lower than in Secondary APS (65%, p <0.005). In UT, aPE were mainly of IgM isotype like in Primary APS and they were found alone whereas in SLE they were always associated with classical antiphospholipid antibodies. No significant association was found between any isotype of aPE and a site of thrombosis in UT as well as in APS. In conclusion, this study demonstrates an increase of the prevalence of aPE in patients with unexplained thrombosis. Thus, aPE investigation appears to be of interest in UT and their persistent presence could define a biological variant of APS.

Introduction: Auto-immune thrombocytopenic purpura is associated with platelet anti-glycoprotein antibodies, particularly with anti-GPIIb/IIIa complex. Persistence of these antibodies sometimes leads to acquired auto-immune thrombopathy., Exegesis: We report the case of a woman treated by splenectomy for auto-immune thrombocytopenic purpura, who developed 5 years later an ecchymotic syndrome despite normal platelet count. High bleeding time and platelet aggregation defect in vitro were evidenced. Following the initial thrombocytopenia, anti-glycoproteins GPIIb/IIIa with lupus anticoagulant and benign monoclonal gammapathy were noticed. Platelet controls showed that hypoaggregant activity was secondary to the persistence of anti-GPIIb/IIa antibodies., Conclusion: This acquired auto-immune thrombopathy simulating Glanzmann's thrombasthenia was secondary to the persistence of platelet anti-glycoproteins GPIIb/IIIa.

To investigate the prevalence, significance, and specificity of IgG isotype anti-beta 2-Glycoprotein I antibodies (a-beta 2-GPI) in antiphospholipid syndrome (APS), we developed an enzyme-linked immunosorbent assay (ELISA) for the detection of IgG-a-beta 2-GPI and tested sera from 61 patients with autoimmune disorders (AID), 39 patients with APS and 22 patients with systemic lupus erythematosus without APS, 139 patients with various infectious diseases (hepatitis C virus infection, human immunodeficiency virus infection, Q fever, Mediterranean spotted fever, syphilis) and 97 healthy control subjects. Using irradiated plates coated with human beta 2-GPI, we showed that in the sera of patients with AID, optical densities from the coated wells were significantly higher than those from the noncoated ones (p = 0.0001). In this assay, intra-assay and inter-assay variation coefficients ranged between 4% and 10%. Clinical evaluation showed that IgG-a-beta 2-GPI were found in 23 of 61 patients with AID but in only one patient with an infectious disease. The presence of the IgG-a-beta 2-GPI in association with APS (p = 0.005) was statistically significant with high specificity (98%) and positive predictive value (87.5%) but with low sensitivity (54%), and was significantly associated with venous thrombosis (p = 0.0025). In addition, the IgG-a-beta 2-GPI levels were highly correlated with those of anticardiolipin antibodies (aCL) (p < 0.001). In contrast to a-beta 2-GPI, aCL were found with a high prevalence (40%) in patients with infectious diseases. Because of their high specificity, anti-beta 2-GPI antibodies appear to be useful tools in the evaluation of the risk of APS. However, because of their low sensitivity, their detection needs to be associated with that of aCL.

The antisynthetases syndrome associates lung disease, polymyositis, Raynaud's phenomenon and frequently polyarthritis. Anti-amioacyl-tRNA synthetase antibodies are present. The most common is anti-Jol. We report a case with severe arteritis of the finger with multiple zones of skin necrosis which responded favourably to corticosteroids given in bolus and cyclophosphamid. Finger arteritis, in addition to classical Raynaud's phenomenon, may be a new manifestation of the anti-synthetases syndrome.

In connection with three observations, we discuss clinical and biological characteristics of "acquired haemophilia". We insist on therapeutics problems: in all the cases corticosteroid and or immunosuppressive drugs are indicated: haemorrhagic accidents treatment depend on the clinical state severity and on the inhibitor concentration: indications of substitutive therapy, plasmapheresis and activated concentrate are considered.

Introduction: ANCA vasculitis may involve the skin and develop slowly without specific histology, and without autoantibodies. Case report: We report a 50-year-old woman who experienced bilateral mastectomy because of ulcero-necrotic, non-specific inflammatory cutaneous lesions of the breasts. First considered by others as a malinger patient, she developed oto-neurological lesions leading to the diagnosis of Wegener's granulomatosis. Five years later, specific antibodies of the disease were present. Conclusion: Cutaneous involvement by ANCA vasculitis can be isolated for a long time. Physicians must have a high degree of suspicion to avoid diagnostic delay of ANCA vasculitis. [ABSTRACT FROM AUTHOR]