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

The objective of the study was to identify the causes, outcome and prognosis of severe illness in patients with systemic lupus erythematosus (SLE) requiring intensive care unit (ICU) care in a University Hospital over a five-year period. The design was a cohort study. Forty-eight SLE patients requiring ICU management over a five-year period (January 1997-December 2001) were studied prospectively. Of 48 patients, 14 (29.2%) died, predominantly with multiorgan dysfunction syndrome (MODS). Patients whose APACHE II score was equal to or greater than 20 had higher mortality than those with APACHE score below 20 (60 versus 7.1%; and P < 0.01). All the 18 patients whose health status rated as 'good' survived, while 46.7% of 30 patients whose health rated as 'poor' died (P < 0.01). Patients who had thrombocytopenia associated with sepsis and/or disseminated intravascular coagulopathy (DIC) had the highest mortality (75%, five-year survival). In conclusion, SLE patients admitted to the ICU had a lower mortality rate than some of the previous reports. Patients with SLE with high APACHE score, > or =20, poor health status, thrombocytopenia and multiorgan dysfunction syndrome had poor prognosis in the ICU.
Lupus 2004
PMID:Outcome of patients with systemic lupus erythematosus in intensive care unit. 1535 26

The antiphospholipid antibody syndrome (Lupus anticoagulans syndrome) is a rare form of coagulopathy due to the presence of autoantibodies against phospholipids or phospholipid-binding protein cofactors that can lead to vascular thrombosis. We report the case of a 57-year-old female patient presenting with decompensated duodenal stenosis due to a pancreatic tumor. Perioperative testing of coagulation markers revealed with 26 % a strongly decreased Quick-Test and with 81.4 s a prolonged partial thromboplastin time that persisted despite intravenous application of 80 mg vitamin K (Konakion) and 10 units of fresh frozen plasma. Subsequent screening for common causes of thrombophilia revealed antiphospholipid antibodies. Consequently, low molecular weight heparin (Dalteparin-Natrium) was administered perioperatively while a gastroenterostomy with entero-enterostomy was performed with uneventful postoperative course. With this presentation and an analysis of the contemporary literature we would like to discuss different aspects of Lupus anticoagulans syndrome.
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PMID:[Paraneoplastic lupus anticoagulans syndrome]. 1610 64

Acquired hemophilia is a rare coagulopathy in adults, associated with bleeding complications. Although the etiology of this disorder remains obscure, an autoimmune mechanism produces the development of autoantibodies against factor VIII. About half of cases are associated with other conditions, mainly post-partum, underlying cancer, autoimmune disease. An 81-year-old male was admitted to the hospital with extensive hematomas (neck, chest, arms and lower limbs). There was no family or personal history of congenital bleeding diathesis. He had chronic bronchitis and cerebrovascular disease; no drugs had been used during the month prior to noted symptoms. Laboratory parameters revealed: hemoglobin 10.9 g%, normal platelet count and white blood cells, prolonged activated partial thromboplastin time (98 s), with normal prothrombin time and fibrinogen concentration. An activated partial thromboplastin time mixing study did not show any correction, suggesting a coagulation inhibitor. Lupus anticoagulant and anticardiolipin antibodies were negative. Biochemical, immunological tests and tumor markers were normal. Thoracic and abdominal computed tomographic scan did not reveal pathological images or hematomas. Analysis of clotting factors revealed decreased factor VIII (< 2%) and elevated factor VIII inhibitor (55 Bethesda units). Idiopathic acquired hemophilia diagnosis was made. Red blood cell transfusion and human factor VIII (2000 U/day for 7 days) infusion were initiated, intravenously with methylprednisolone. A progressive improvement in clinical conditions and laboratory parameters was observed. After 18 days the patient was discharged and treated with prednisone. At follow-up control the clinical conditions and laboratory parameters were normal.
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PMID:[Acquired factor VIII hemophilia in a geriatric patient]. 1625 Jan 80

We describe two previously healthy children who had multiple ecchymoses several days after acute infection. In both cases, the prothrombin time (PT) and the activated partial thromboplastin time (APTT) were prolonged. Further examinations revealed the presence of lupus anticoagulant (LA), phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT), and low serum complement. In both cases, we confirmed the presence of a serum immune complex. The patients' symptoms improved spontaneously within 1 week, and all laboratory data normalized within several months. We also describe another asymptomatic case positive for LA and aPS/PT presumably associated with cytomegalovirus infection. The prevalence of transient antiphospholipid antibodies associated with viral infections in children must be much higher than we expected. We have to take it into consideration when we see abnormal coagulation results, but the occurrence of significant bleeding symptoms is rare.
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PMID:Transient antiphospholipid antibodies associated with acute infections in children: a report of three cases and a review of the literature. 1655 44

Familial Mediterranean fever (FMF) patients in clinical remission are reported to have increased baseline inflammation. Normal function of the natural anticoagulant pathways is particularly needed in diminishing inflammatory responses. In the presence of subclinical inflammation, natural anticoagulant response may be exaggerated. We aimed to observe the anticoagulant-procoagulant status in attack-free FMF patients. Twenty-seven FMF patients diagnosed in accordance with Tel-Hashomer criteria, and 26 healthy controls were included. All patients were attack-free under regular colchicine treatment. Amyloidosis, autoimmunity, accompanying liver and renal disease, and vasculitis were excluded. Predisposing factors for thrombosis were not present. Acute phase reactants (APRs), anticardiolipin antibody positivity, prothrombin time (PT), activated prothrombin time, thrombin time (TT) and d-dimer, protein C activity, activated protein C resistance, free protein S, antithrombin, lupus anticoagulant, human prothrombin fragment F 1 + 2, and human thrombin/antithrombin III complex were analyzed for all subjects. APRs were comparable with controls. Autoimmune markers were negative in all. Anti-streptolysin titers were significantly different than the control group. PT, TT, protein C activity, and F 1 + 2 levels were significantly different from those of healthy controls. Shortened PT and TT, decreased protein C activity vs increased levels of F 1 + 2 suggested a hypercoagulable state in our patients. The hypercoagulable state detected in FMF patients suggests that screening with abnormal coagulation tests may be beneficial for tracing the future consequences of subclinical inflammation in these patients. Studies covering larger groups of patients are needed to verify the currently observed hypercoagulable status in FMF.
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PMID:Hypercoagulability: interaction between inflammation and coagulation in familial Mediterranean fever. 1672 92

Necrotizing fasciitis is an uncommon but life-threatening complication in immunocompromised hosts. We reported four patients with rheumatic diseases complicated by necrotizing fasciitis and reviewed 14 others from literature search. Most patients were on corticosteroid treatment. Septic shock, disseminated intravascular coagulopathy and acute renal deterioration were common giving rise to an overall mortality rate of 27.8%. Septic arthritis may also complicate the condition. Statistical analysis on the series showed the lack of major surgical debridement as the only risk factor associated with increased mortality (RR 7.5, 95% CI 2.1-27.3, P = 0.01). Timely debridement of necrotic tissue is important for reducing mortality.
Lupus 2006
PMID:Necrotizing fasciitis in rheumatic diseases. 1683 Aug 85

Brain magnetic resonance imaging (MRI) studies in migraine patients have demonstrated lesions consisting of focal regions of increased signal intensity within the white matter. Antiphospholipid antibodies are known to have a role in many diseases including migraine. The aim of the present study was to ascertain the relationship between MRI-visualized cerebral focal hyperintense lesions and serum antiphospholipid antibody levels, as well as blood coagulation parameters in migraine patients. One hundred and two (77 females, 25 males, mean age 33.8 +/- 11.1) consecutive migraine patients and a control group of 94 (70 females, 24 males, mean age 33.2 +/- 10.8) healthy subjects were enrolled. All individuals underwent brain MRI. Complete blood examinations, autoantibodies, antiphospholipids antibodies including anticardiolipin and lupus anticoagulant (aCL, LAC), antithrombin III, Protein C and S serum levels were ascertained in the subjects who presented white matter lesions on MRI. Twenty-seven (26.4%) migraine patients and six (6.3%) healthy subjects in the control group showed focal regions of increased intensity signal within cerebral white matter (odds ratio 5.3, 95% CI: 1.98-16.36). In migraine patients with white matter lesions, antiphospholipid antibodies were not detected and serum levels of antithrombin III, and proteins C and S were normal. White matter lesions in migraine patients are fairly common. This finding is not associated with antiphospholipid antibodies or abnormal coagulation parameters. The significance of such lesions at present remains unclear.
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PMID:Brain MRI white matter lesions in migraine patients: is there a relationship with antiphospholipid antibodies and coagulation parameters? 1711 21

Lupus anticoagulant syndrome (LAS) is a form of coagulopathy with protracted PTT and an elevated risk of thromboembolic events, which occasionally occurs as a paraneoplastic phenomen in the presence of tumors. It is diagnosed by specific coagulation tests that identify lupus anticoagulant antibodies. We present the case of a 57-year-old woman with a coagulopathy caused by lupus anticoagulant antibodies as a paraneoplastic phenomenon in the course of a relapse of clear cell renal cell carcinoma in the area of the ipsilateral adrenal gland 18 months after tumor nephrectomy. After adrenalectomy with concomitant administration of low-molecular-weight heparin the PTT became normal and the diagnostic tests for lupus anticoagulant antibodies became negative.
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PMID:[Coagulopathy resulting from lupus anticoagulant antibodies as a paraneoplastic phenomenon in renal cell carcinoma relapse]. 1792 18

The pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) may be related to autoantibody-mediated neural dysfunction, vasculopathy and coagulopathy. We encountered an NPSLE patient whose brain showed characteristic diffuse symmetrical hyperintensity lesions in the cerebral white matter, cerebellum and middle cerebellar peduncles on T2-weighted magnetic resonance (MR) images. In this study, we investigated all the antigens that reacted strongly with autoantibodies in this patient's serum by two-dimensional electrophoresis (2DE), followed by western blotting (WB) and liquid chromatography-tandem mass spectrometry (LC-MS/MS) using rat brain proteins as the antigen source. As a result, we identified four antigens as beta-actin, alpha-internexin, 60 kDa heat-shock protein (Hsp60) and glial fibrillary acidic protein (GFAP). There are several reports on the detection of anti-endothelial cell antibodies (AECAs) in an SLE patients. Recently, one of the antigens reacting with AECAs in SLE patient's sera has been identified as human Hsp60. We speculated that the abnormal findings on brain MR images of our patient may be due to impairment of microcirculation associated with vascular endothelial cell injury mediated by the antibody against Hsp60. This proteomic analysis is a useful tool for identifying autoantigens in autoimmune diseases involving autoantibodies.
Lupus 2008 Jan
PMID:Proteomic analysis of autoantibodies in neuropsychiatric systemic lupus erythematosus patient with white matter hyperintensities on brain MRI. 1808 78

Azathioprine (AZA) is a commonly used immunosuppressant for systemic lupus erythematosus (SLE). Myelosuppression is a serious adverse reaction due to AZA and its metabolites. Thiopurine S-methyltransferase (TPMT) is the rate-limiting enzyme. Variations of TPMT enzyme activity may be responsible for myelosuppression. However, a correlation between certain mutant alleles of low TPMT enzyme activity and myelotoxicity has also been suggested as a factor. We describe herein a case of AZA-induced severe myelosuppression associated with TPMT*3C heterozygous mutant allele in a SLE patient. The patient presented with pancytopenia, sepsis, typhlitis and disseminated intravascular coagulopathy after a short period of AZA therapy. The patient had low TPMT activity and TPMT*3C genotype. Measurement of TPMT activity and determination of TPMT variant allele may identify patients at risk for AZA-induced myelosuppression.
Lupus 2008 Feb
PMID:Azathioprine-induced fatal myelosuppression in systemic lupus erythematosus patient carrying TPMT*3C polymorphism. 1825 Jan 37


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