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)

The presence of a lupus anticoagulant (LA) is paradoxically associated with a high incidence of arterial and venous thrombosis. In a patient with a lupus-like systemic disease, having received phenindione for 11 years, LA was discovered in association with recurrent deep venous thrombosis, a right atrial thrombus, coronary occlusion, arterial hypertension, thrombopenia, and anticardiolipin antibodies without anti-DNA antibodies. Renal cortical ischemia was detected by a tomographic scan. Renal biopsy showed glomerular ischemia and diffuse interstitial fibrosis. After a one-year anticoagulant and steroid therapy, LA has disappeared despite a high level of anticardiolipin antibodies, and renal function remains normal.
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PMID:[Renal cortex ischemia, right atrial thrombosis and coronary occlusion in anti-phospholipid antibody syndrome]. 251 17

Antiphospholipid antibodies (APAs) may be identified in the laboratory by using either coagulation studies or solid-phase immunologic assays (ELISA; RIA). These methodologies do not necessarily evaluate the same antibody; consequently, it is appropriate to screen a patient's plasma by utilizing both assays. APAs have been associated with a variety of obstetrical complications including recurrent spontaneous abortion, intrauterine fetal death, early onset preeclampsia, deep vein thrombosis, and postpartum serositis syndrome. The Kaolin Clotting Time appears to be the most sensitive coagulation test for identifying the lupus anticoagulant. However, preliminary studies would suggest the presence of anticardiolipin antibodies as detected by solid-phase assays are more sensitive and predictive of the clinical course. Although there are no prospective trials to analyze treatment of patients with APA, preliminary data suggest the use of prednisone in combination with aspirin significantly improves the probability of delivery of a viable infant. In addition, heparin, intravenous gammaglobulin, and exchange plasmaphoresis have all been tried with varying degrees of success in individual patients in small series.
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PMID:Antiphospholipid antibodies and reproduction. 251 82

Since most patients with thrombophilia in Israel are referred for diagnosis to our center, it was possible to estimate the relative frequency of the hereditary disorders leading to thrombophilia. 107 unrelated patients were evaluated over 4 years. Diagnoses were established in 23 patients (21.5%) while in 84 (78.5%) no abnormality was detected. Antithrombin III deficiency was found in 8 patients (7.5%), dominant protein C deficiency in 6 (5.6%), recessive homozygous protein C deficiency in 1, protein S deficiency in 3 (2.8%) and dysfibrinogenemia in 1. Four additional patients (3.7%) had a lupus anticoagulant. The frequency of deep vein thrombosis and pulmonary embolism was similar in patients with and without a definite diagnosis. Thrombosis of visceral or cerebral vessels and a positive family history were more frequent among patients in whom a definite diagnosis was made. In both groups there was a substantial lag between the time of presentation of the first thrombotic episode and the time of evaluation. Since the number of referred patients with thrombophilia has gradually increased over the period of the study, it is at present impossible to establish the prevalence of the various hereditary disorders leading to thrombophilia in the population.
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PMID:The relative frequency of hereditary thrombotic disorders among 107 patients with thrombophilia in Israel. 252 86

A 34 year old man with a past history of deep vein thrombosis, positive lupus serology, and high titres of anticardiolipin antibodies developed acute adrenal insufficiency. Association of anticardiolipin antibodies with adrenal insufficiency has not been previously reported.
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PMID:Acute adrenal insufficiency as a manifestation of the anticardiolipin syndrome? 238 70

Fifteen patients (9 females and 6 males) with the "lupus type" circulating anticoagulant have been studied. The underlying disease was an auto-immune disorder in 11 cases and a malignant hemopathy in 4 cases. The manifestations frequently associated with the lupus inhibitor, such as thrombosis, thrombocytopenia and false-positive VDRL test were analysed. Hemorrhagic syndrome occurred only when thrombocytopenia or acquired abnormality of Willebrand's factor was present. Thrombotic events (8 cases) were frequent. Deep venous thrombosis was complicated with pulmonary embolism in 4 patients. Platelet abnormality, decreased fibrinolytic response or acquired Willebrand's syndrome were found in all patients with a thrombotic event. These different manifestations followed diverging courses in some patients with persistent thrombocytopenia although the anticoagulant had disappeared in 3 cases, negativation of the false-positive VDRL test while the anticoagulant remained unchanged in 1 case, occurrence of a thromboembolic episode although the anticoagulant had disappeared in 1 case.
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PMID:[Course of the manifestations associated with the antiprothrombinase circulating anticoagulant]. 293 5

The patient, a 44-year old woman with systemic lupus erythematosus, (SLE), developed infarction of the bowel and spleen after occlusion of the inferior mesenteric and splenic arteries, necessitating colectomy and splenectomy. She had had previous cerebral thromboses and a lower limb deep vein thrombosis. Histological examination of the involved vessels showed the presence of thrombus only with the total absence of any vasculitis. The patient demonstrated antibodies to phospholipid - the "lupus anticoagulant" (LA) and antibodies to cardiolipin in serum, both strongly associated with thromboses.
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PMID:Arterial occlusion causing large bowel infarction--a reflection of clotting diathesis in SLE. 308 70

Two patients with the lupus anticoagulant exhibited unusual cutaneous manifestations. They both fulfilled four criteria for systemic lupus erythematosus and had experienced deep venous thrombosis. The first patient suffered from a leg ulcer that resembled a pyoderma gangrenosum. The second patient presented erythematous and purplish macules on the fingertips. The histologic studies showed only microthrombosis in the dermal vessels without vasculitis, although such lesions in systemic lupus erythematosus are usually attributed to vasculitis. The association of these cutaneous lesions with lupus anticoagulant has never been reported. It is likely that this association is not fortuitous. After a review of the literature, it seems possible to individualize a new syndrome characterized by the presence of a subgroup of antiphospholipid antibodies. Thrombosis, spontaneous abortions, neurologic manifestations, pulmonary hypertension, positive results of a Coombs' test, and thrombocytopenia can be included in this syndrome, which overlaps with systemic lupus erythematosus. Certain cutaneous symptoms are associated with the presence of lupus anticoagulant or other antiphospholipid antibodies: leg ulcers, distal cutaneous ischemia, widespread cutaneous necrosis, and livedo. They can be considered as the dermatologic manifestations of this syndrome.
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PMID:Cutaneous manifestations associated with the presence of the lupus anticoagulant. A report of two cases and a review of the literature. 309 56

A lupus inhibitor paradoxically prolongs phospholipid-dependent coagulation assays, but may increase risk of thromboembolism. We studied seven patients with cerebral infarcts and one with TIA who had lupus inhibitor. The average age at onset of cerebral ischemia was 41 years. Three patients had multiple cerebral ischemic events. The activated partial thromboplastin time was longer than that of controls, but usually within normal limits. Other abnormalities included biologic false-positive VDRL, antinuclear antibodies, thrombocytopenia (three patients each), and deep vein thrombosis (two patients).
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PMID:Cerebral infarct, TIA, and lupus inhibitor. 309 32

Lupus anticoagulant, an immunoglobulin that prolongs the partial thromboplastin time, has been associated with thrombotic events, including deep venous thrombosis, pulmonary emboli, and Budd-Chiari syndrome. In this report, primary sclerosing cholangitis was diagnosed in a man with a 10-year history of multiple thrombotic events related to a circulating lupus anticoagulant. Progressive jaundice and pruritus developed, and sclerosing cholangitis was confirmed by direct cholangiography. Sclerosing cholangitis is the second hepatobiliary disease reported in association with a lupus anticoagulant.
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PMID:Primary sclerosing cholangitis in the presence of a lupus anticoagulant. 309 67

Recent reviews have suggested a higher frequency of the lupus anticoagulant or related antiphospholipid antibodies in patients with systemic lupus erythematosus (21% to 65%) than was found in earlier studies (6% to 18%). In our study of 60 consecutive patients, we found the frequency of the lupus anticoagulant by Russell viper venom time was 6.7% (95% confidence interval, 16.2 to 1.8) and by anticardiolipin antibody assay was 25% (95% Cl, 37.0 to 15.7), compared with 0% (p = not significant) and 2.5% (p = 0.002), respectively, in the normal control population. The Russell viper venom time (p = 0.0001 by t-test) and anticardiolipin antibody levels (p = 0.01) were significantly associated with presumed thrombotic events (stroke, deep venous thrombosis, and digital gangrene). No association with miscarriage or pulmonary hypertension was detected. The Russell viper venom time was more specific than the anticardiolipin antibody level in the prediction of past presumed thrombotic events, miscarriage, or pulmonary hypertension (100% compared with 84%, p = 0.01).
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PMID:The frequency of lupus anticoagulant in systemic lupus erythematosus. A study of sixty consecutive patients by activated partial thromboplastin time, Russell viper venom time, and anticardiolipin antibody level. 310 10


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