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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There has been a recent, dramatic surge in interest in antiphospholipid antibodies and associated clinical disorders, especially focal ischemic cerebrovascular disease. Antiphospholipid antibodies are a heterogeneous group of antibodies with varying specificities. Coagulation assays will detect lupus anticoagulants while enzyme-linked immunosorbent assays detect anticardiolipin antibodies. There are numerous potential links between antiphospholipid antibodies and coagulation disorders, including interaction of antiphospholipid antibodies and a cofactor, beta 2-glycoprotein I, which itself is involved in coagulation mechanisms. While the specific mechanism of antiphospholipid antibody-related coagulopathy is unknown, it is clear that antiphospholipid antibodies are associated with an immune-mediated prothrombotic state. Patients with the highest titers of IgG antiphospholipid antibodies have a relatively high risk of recurrent thrombotic events, especially stroke, deep venous thrombosis, and spontaneous abortion. Because of limited controlled, prospective data, current therapy remains empiric and directed at coagulation mechanisms, immune mechanisms, or both.
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PMID:Cerebrovascular disease with antiphospholipid antibodies: immune mechanisms, significance, and therapeutic options. 896 22

A 17-year-old girl was admitted to our department with a hemorrhagic syndrome due to a serious coagulopathy; prothrombin time (PT) INR was 2.46 and the activated partial thromboplastin time (aPTT) ratio 3.46. Coagulation tests with pooled normal fresh plasma did not correct aPTT because of a coagulation inhibitor, and only partially corrected PT. Factor II activity reached only 5%. Diluted Russell viper venom tests (dRVVT) and kaolin clotting time (KCT) of patient plasma (PP) and of a mixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). The level of factor II antigen was 10%. We diagnosed systemic lupus erythematosus (SLE) with a rare acquired hypoprothrombinemia-LA syndrome (HLAS). The patient was treated with corticosteroids and high-dose Ig and a normal PT value was re-established.
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PMID:Severe bleeding due to acquired hypoprothrombinemia-lupus anticoagulant syndrome. Case report and review of literature. 923 88

We examined thrombophilic mechanisms and outcome in 54 patients with deep-vein thrombosis (DVT), who were otherwise apparently healthy and aged < or = 50 years. Patients were followed up 6 years (median) after a confirmed first DVT between 1987-1992 with no known predisposing illnesses. Patients were traced through the hospital registry and compared with 25 matched controls. Tested thrombophilic mechanisms were either genetic (activated protein C [APC] resistance; anti-thrombin III deficiency [ATIII]; protein C or protein S deficiency [PC, PS]) or acquired (lupus anti-coagulant [LAC]/anti-cardiolipin antibodies [ACA]; subsequent diagnosis of cancer). Twenty-nine DVT patients attended for full studies. The remaining 25 were interviewed by phone and none had a reported neoplastic disease, confirmed by their hospital records and the National Cancer Registry. These patients' demographics, risk factors and subsequent course were similar in all respects to the studied group. In the control group, APC resistance was the only coagulopathy found (1/25, 4%), and it was also the most common abnormality among DVT patients (8/29, 28%) (p = 0.009). Three DVT patients had LAC/ACA (10%) and one each, ATIII, PC and PS deficiencies (3.3% each). No malignancy was encountered during a follow-up of 7.9 +/- 5.7 years. Circumstantial risk factors were found in 52% of the patients, 21% had a family history of DVT, and 41% had recurrent DVT. These characteristics were not significantly different when DVT patients with and without coagulopathy were compared.
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PMID:Causes and outcome of deep-vein thrombosis in otherwise-healthy patients under 50 years. 930 63

We report a patient with clinical, biochemical and immunological indices suggestive of autoimmune hepatitis with marked transaminasaemia, raised immunoglobulins and positive anti-nuclear and anti-smooth muscle antibodies. A coagulation screen revealed a transient, markedly increased, activated, partial thromboplastin time and a normal prothrombin time, with elevated levels of anticardiolipin antibodies and the presence of lupus anticoagulant, indicating the presence of antiphospholipid antibodies. Subsequent histology confirmed moderate hepatitis with piecemeal necrosis and fibrosis. Appropriate autoimmune therapy was commenced. This presentation illustrates the rare association of anti-phospholipid antibodies with autoimmune chronic active hepatitis presenting with a temporarily abnormal coagulation screen.
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PMID:Autoimmune chronic active hepatitis associated with the presence of antiphospholipid antibodies. 958 93

Autoantibodies directed to phospholipids or to phospholipid binding proteins are now studied using a growing number of laboratory tests. However, the history of the interest in this area goes back to the identification of the so-called false positive reactions in the non-treponemal serological test for syphilis (STS) and to the subsequent description of an in vitro coagulation defect called lupus anticoagulant (LAC). In the 1980s the introduction of the anticardiolipin antibody (aCL) immunoassay was a determining factor in the definition of the antiphospholipid syndrome (APS). In addition, lupus prone mice spontaneously producing aCL antibodies and normal mice passively infused with these antibodies provided useful models for the study of the pathogenic role of aPL. When in 1990 a phospholipid binding protein (beta 2glycoprotein I, beta 2GPI) was identified as the cofactor required for aCL antibody binding, the true antigenic target of the antibodies was first discussed. Soon afterwards an anti-beta 2GPI ELISA was developed that has proved to be of great clinical significance. We will discuss here the similarities and differences between these various assays (LAC, aCL, and anti-beta 2GPI), focusing on their specificity, sensitivity and practical applications.
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PMID:Anticardiolipin and anti-beta 2glycoprotein I immunoassays in the diagnosis of antiphospholipid syndrome. 970 18

Pulmonary hemorrhage is a rare, but serious manifestation of systemic lupus erythematosus (SLE). Herein, we report 13 cases of severe pulmonary hemorrhage in SLE. Hemoptysis was present in 11 patients. All thirteen patients had active nephritis and were in the stage of nephrotic syndrome. A majority of the patients had neuropsychiatric manifestations and coagulopathy including thrombocytopenia or lupus anticoagulant. All episodes of pulmonary hemorrhage occurred after large dose of corticosteroid had been administered in treating nephritis. Recurrent pulmonary hemorrhage was noted in four patients. Ten (77%) of the 13 patients finally died. Respiratory failure was the main cause of death. Our observation suggests that active nephritis with hypoalbuminemia is a major risk factor for severe pulmonary hemorrhage in SLE patients and that high dose corticosteroid use can not prevent the occurrence of severe pulmonary hemorrhage in SLE.
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PMID:Clinical experience of 13 cases with severe pulmonary hemorrhage in systemic lupus erythematosus with active nephritis. 975 70

With the use of low-dose heparin, fetal survival of aPL pregnancies is 70-80%, but prematurity and intrauterine growth restriction are common. It is likely, but not proven, that dysregulated placental coagulation and resultant vasculopathy are the cause of fetal loss. Details of dysregulated coagulation remain to be described. Opportunities remain to determine the role of coagulopathy in repeated pregnancy loss, identify a critical event or window to which intervention might be directed, identify maternal (and fetal) characteristics other than aPL that determine fetal loss, describe toxicity profiles of current treatments, develop more specific, less toxic therapies, and describe long-term fetal and maternal outcomes.
Lupus 1998
PMID:Pregnancy loss and antiphospholipid antibodies. 981 80

A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).
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PMID:The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports. 1022 60

We describe two patients whose initial presentation of systemic lupus erythematosus (SLE) was accompanied by haemorrhagic episodes and significant coagulopathy. Further investigation demonstrated positive lupus anticoagulant and decreased Factor II (prothrombin) activity. Both patients were diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LAC-HPS) as a result of non-neutralizing antibodies directed against Factor II. LAC-HPS is a rare clinical entity that can occur in association with SLE, transient viral infections, drug reactions or even in healthy individuals. Mixing studies, which can be affected by other coagulation factor inhibitors, play an important role in the diagnosis of LAC-HPS. Factor VII level was decreased in the second patient, a finding that has not previously been reported in association with SLE. In both patients, bleeding stopped promptly and coagulation studies improved significantly with high dose corticosteroids. We discuss the pathogenesis, diagnosis and management of LAC-HPS in patients with SLE.
Lupus 1999
PMID:Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus: report of 2 cases and review of literature. 1048 36

A 14-year-old man was admitted with sudden onset of paralysis in his lower extremities paralysis and of sensory loss below Th10 level. On admission, linear high intensity signals was seen in the spinal cord from Th 8 to Th 12 level on thoracic MRI T2 weighted image. Laboratory data on admission indicated existence of lupus anticoagrant in the patient's serum. Systemic lupus erythematodes was negative in his past history. The diagnosis of transverse myelitis caused by primary antiphospholipid syndrome was made accordingly. Plasmapheresis was performed to remove the lupus anticoagrant. After plasma pheresis coagulopathy was normalized with disappearance of the lupus anticoagrant. The lesion of the spinal cord is too extensive to be caused by single obstruction of any one blood vessel branch of the spinal cord. The transverse myelitis may be aggravated by the direct invasion of the lupus anticoagrant into nervous tissue from the vasculature whose blood brain barrier had been compromised by intravascular coagulation of this substance.
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PMID:[A case of transverse myelitis caused by primary antiphospholipid antibody syndrome]. 1061 66


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