Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are closely related, but each has it own characteristic vasculopathy: vasculitis in SLE and thrombosis in APS, and either may be a serious or life threatening complication. When a patient has SLE associated with APS, systemic and cerebral vasculitis may coexist with disseminated coagulopathy. We report this complication in a 60-year-old woman who died from stroke and myocardial infarction, an occurrence seldom documented in the literature.
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PMID:Systemic and cerebral vasculitis coexisting with disseminated coagulopathy in systemic lupus erythematosus associated with antiphospholipid syndrome. 859 66

The reactive hemophagocytic syndrome is a condition characterized by systemic proliferation of benign hemophagocytic histiocytes, fever, cytopenia, abnormal liver function, and frequently coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections; a wide spectrum of malignant neoplasms; some miscellaneous disorders; and phenytoin. Disseminated strongyloidiasis is reported in a patients with systemic lupus erythematosus treated with corticosteroids in whom a reactive hemophagocytic syndrome developed and who finally died. This reactive hemophagocytic syndrome is reported for the first time in strongyloidiasis and may not have been recognized in former patients.
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PMID:Case report: reactive hemophagocytic syndrome associated with disseminated strongyloidiasis. 868 29

Coagulation disorders usually confront the emergency physician as bleeding episodes or as abnormalities of laboratory tests. Bleeding has to be treated aggressively, while pathological coagulation tests should be related to a more differentiated diagnosis at first. The most common causes of acquired coagulation disorders are liver disease, vitamin K deficiency, and disseminated intravascular coagulation (DIC). More rarely, inhibitors, external factors such as drugs or extracorporeal circulation, or other diseases such as amyloidosis are present. Since localized hemorrhage is the most common bleeding source in liver disease, endoscopic and surgical therapeutic measures, respectively, are warranted. Careful and balanced substitution therapy according to laboratory findings should be initiated simultaneously and should consist of fresh frozen plasma (FFP), which contains all components of the coagulation system physiologically balanced. Prothrombin complex concentrates should be used in emergency situations only, keeping their potential hazards in mind. Adequate vitamin K substitution is indicated in liver disease as well as in coagulopathy due to vitamin K deficiency. Management of DIC primarily consists of aggressive treatment of the underlying disease. Substitution therapy is difficult and should be carefully monitored by the adequate laboratory tests. FFP is the adequate source of both procoagulants and inhibitors but may cause certain problems. Heparin therapy can be beneficial but is not recommended generally. Antithrombin III substitution cannot be assumed as established therapy so far. Inhibitors can lead to bleeding, but the most common inhibitor, lupus anticoagulant, rather predisposes to thrombosis. In bleeding patients with inhibitors against single clotting factors, treatment consists of adequate substitution before initiating the diagnostic workup.
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PMID:Management of acquired coagulation disorders in emergency and intensive-care medicine. 871 94

We reviewed 44 cases of ischemia and infarction of the spinal cord at two university hospitals. Three patients experienced transient ischemic attacks. Etiologies of completed strokes were diverse and included rupture and surgical repair of aortic aneurysms, aortic dissection, aortic rupture and thrombosis, global ischemia, anterior spinal artery embolism, repair and thrombosis of spinal arteriovenous malformations, hematomyelia, epidural hematoma, cervical osteophytosis, celiac plexus block, systemic lupus erythematosus, coagulopathy, and decompression sickness. Motor function improved in 12 patients, was substantial in only one, and occurred largely within the first 2 to 4 weeks. Favorable ambulatory outcome correlated with improving neurologic examinations and relatively preserved strength in hip abductors and knee extensors. More extensive deficits without initial improvement portended a more severe prognosis. Autonomic dysfunction, pain, paresthesia, and depression were common and impeded recovery in some patients. The mean level of deficit was at T-8 and in cases of global ischemia was at T-9, which leads us to dispute the classical view of a midthoracic watershed zone of ischemic vulnerability near T-4.
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PMID:Spinal cord infarction: etiology and outcome. 915 13

Spontaneous liver rupture is uncommon, difficult to diagnose and carries a universally high mortality. It has been well documented to occur as a complication of primary or secondary hepatic malignancy. Similarly, there are 28 cases of ruptured haemangiomata described in the world literature. It is also well described in severe pregnancy-induced hypertension and is said to carry a mortality of 18% for patients treated by packing and drainage of the haematoma and 75% for patients treated with liver resection. Two female patients aged 60 and 61 presented to our accident and emergency department. One had a history of hypertension only and the other a history of a bleeding diathesis from the lupus anticoagulant. Both presented with hypotension and abdominal pain and both were diagnosed by abdominal CT scan. One was treated with hepatic artery ligation and tamponade and the other with liver resection and correction of the coagulopathy. Neither had any evidence of a ruptured hemangioma or tumor at laparotomy or on histological examination, and both are alive and well. The conclusions to be drawn from this review and our own recent experience is that the treatment of choice for ruptured haemangiomata is liver resection and, for rupture during pregnancy, is tamponade with packs and evacuation of the haematoma. Hepatic arteriography and embolisation, if possible, is a useful adjunct. Correction of any coagulopathy is essential. We can only speculate that the aetiology in our patients was uncontrolled hypertension in one and coagulopathy in the other.
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PMID:Two cases of spontaneous liver rupture and literature review. 880 90

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


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