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)

Our objective was to define the renal involvement in primary antiphospholipid syndrome (APS). We studied 20 patients with primary APS. Fourteen were women, mean age 34.4 years. None met ARA criteria for systemic lupus erythematosus. All patients underwent complete renal function studies. The presence of hypertension was also investigated. Renal disease was found in 5 patients, and was characterized by proteinuria, hypertension and renal failure. Kidney biopsy was performed in these 5 patients, showing thromboses of the microvasculature, mesangiolysis, mesangial interposition, electron lucent subendothelial material and ischemic obsolescence of glomeruli. Arterioles showed luminal narrowing due to medial hypertrophy, mucoid thickening of the intima, thrombosis and fibrosis. We found renal disease in 25% of our patients with primary APS. Biopsy findings were consistent with a thrombotic microangiopathy involving both arterioles and glomerular capillaries.
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PMID:Renal involvement in primary antiphospholipid syndrome. 815 77

Circulating lupus anticoagulant (LA) is associated with thrombosis in large and small vessels. To determine how often the presence of LA is associated with thrombosis within the renal microcirculation, 33 patients with systemic lupus erythematosus (SLE), renal dysfunction, and LA were identified over a 25-year period (LA group) and 32 patients with renal SLE but with normal gross coagulation screen were matched for age, sex, and biopsy timing (C group). Prevalences of serositis, neuropsychiatric illness, leukopenia, thrombocytopenia, hemolysis, anti-DS-DNA elevation, and complement reduction were similar. Arthritis was less and biologic false-positive (BFP) syphilis serology more common in LA. More LA patients had thrombotic events (LA 39% v C 13%; P = 0.014); bleeding episodes, including postbiopsy, were similar. At biopsy, hypertension (LA 55%, C 41%), serum creatinine (mean +/- SD: LA 186 +/- 168 mumol/L [2.1 +/- 1.9 mg/dL] v C 150 +/- 168 mumol/L [1.7 +/- 1.9 mg/dL]) and proteinuria (LA 2.6 +/- 3.1 g/24 h v C 3.1 +/- 2.7) were similar. Lesions by World Health Organization (WHO) class, activity, and chronicity indices, as well as immunofluorescence (IF) and electron microscopy (EM) findings, were not significantly different. Occlusive glomerular, arteriolar, and arterial fibrin thrombi, along with varying degrees of renal thrombotic microangiopathy, were seen in five of 33 patients with LA, but zero of 32 C patients (P = 0.053); three of these five patients died soon after biopsy. Overall, mortality was not different between LA and C. We conclude that the majority of patients with SLE, renal dysfunction, and LA exhibit renal morphologic findings indistinguishable from patients without LA. However, a significant minority of LA patients have thrombotic microangiopathy in their biopsy, which is accompanied by a worse prognosis.
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PMID:Lupus anticoagulant in systemic lupus erythematosus: a clinical and renal pathological study. 144 58

The nephrotic syndrome is characterized by proteinuria, hypoalbuminemia and hypercholesterolemia. Hypercholesterolemia is in some cases a risk factor for atherosclerosis in this group of patients. The lipid plasma spectrum was studied in 45 patients with the nephrotic syndrome. Most pronounced changes of the lipid composition of the plasma were revealed in patients with systemic lupus erythematosus and a special form of mesangio-proliferative glomerulonephritis which is characterized by a torpid course and rapid development of chronic renal failure. Plasma atherogenicity was calculated according to the index of plasma atherogenicity. A high atherogenicity index was revealed in patients with an association of the nephrotic syndrome and arterial hypertension. Plasma atherogenicity is determined mainly by the level of high-density-lipoprotein cholesterol.
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PMID:[Lipidemia in the nephrotic syndrome and the atherogenicity of the plasma]. 145 41

From June 1987 to June 1991 at the Belgrade University Children's Hospital 10 patients, 5 males and 4 females, aged 2-16 years, with chronic glomerular disease, were treated with CyA. Seven patients had INS, 2 lupus nephritis and one IgA nephritis. Before initiation of CyA, all but one, were treated with classic immunosuppressive therapy, which had no effect (8/10) and/or had serious adverse effects (9/10). CyA dosage was initiated at 4-6 mg/kg/BW, and was subsequently adjusted to achieve CyA concentrations in blood at range 50-100 ng/ml. Treatment duration was 2-17 months. Patient compliance to CyA therapy was observed in 5/7 INSs: 2 cortico-sensitive (1 with FSGS was cortico-dependent and 1 had frequent relapses) and 3 cortico-resistant patients (2 with FSGS and 1 with minimal histologic changes). After drug withdrawal, only one of the patients who responded, had no relapse. One of the two patients with SLE showed improvement during CyA administration, while no response was observed in the patient with IgA nephritis. Adverse experiences with CyA therapy involved decreased renal function (2/10), arterial hypertension (1/10), hyperbilirubinaemia (1/10), transient LDH increase and hyperuricaemia (1/10).
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PMID:[Cyclosporine in the treatment of glomerular diseases in children]. 146 61

Postpartum hemolytic uremic syndrome (HUS) is described in a woman with a history of spontaneous abortions and both circulating lupus anticoagulant and anticardiolipin antibody (ACA). After termination of her pregnancy because of severe preeclampsia, ACA blood levels increased simultaneously with the onset of a microangiopathic process associated with severe hypertension and renal failure. Plasma exchange resulted in a rapid decline in ACA levels and immediate improvement in her clinical condition. This case strongly suggests an important causal relationship between ACA and postpartum HUS. The possible mechanisms of ACA-related postpartum HUS and the potential role of plasmapheresis in its treatment are reviewed and discussed.
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PMID:Postpartum hemolytic uremic syndrome associated with antiphospholipid antibodies. A case report and review of the literature. 149 77

Known risk factors for coronary artery disease are very common in the Hopkins Lupus Cohort, in spite of the fact that the average patients age is only 38.3 years. Three or more known risk factors were found in 53% of patients. Risk factors for CAD were common even in patients not on a regimen of prednisone therapy during their cohort follow-up. Hypercholesterolemia increased significantly with greater average prednisone dose. Despite the frequency of risk factors, patients' awareness of the risk of CAD was low, with only 16.9% of patients believing they were at high risk for developing CAD within 5 years. In general, awareness of individual risk factors was lower in black than in white patients with SLE. Preventive practices were most commonly addressed towards hypertension. Preventive practices directed against obesity, hypercholesterolemia, and smoking were underutilized. Whether these known risk factors are sufficient in and of themselves to explain the high frequency of CAD in the cohort (8%) or whether they are "enabling" factors acting upon endothelium damaged by immune-complex disease cannot be addressed by this study. However, both further investigation of these risk factors and attention to lifestyle and pharmacologic approaches to risk factor reduction are indicated by this study.
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PMID:Coronary artery disease risk factors in the Johns Hopkins Lupus Cohort: prevalence, recognition by patients, and preventive practices. 152 5

Patients with systemic lupus erythematosus may develop premature atherosclerosis, notably coronary artery disease. A group of 10 patients with peripheral vascular disease presenting with intermittent claudication or gangrene were studied from a group of 563 patients followed prospectively at the Wellesley Hospital Lupus Clinic. These 10 patients were compared with the next lupus clinic patient matched for age and sex, with respect to demographic characteristics and risk factors. The patients and controls did not differ significantly in lupus activity criteria count, partial thromboplastin time, the number with antibody to cardiolipin, number receiving steroids or mean steroid dose, family history of atherosclerosis, hyperlipidaemia, smoking, hypertension or use of oral contraceptives. The risk factors for developing peripheral vascular disease were a longer duration of systemic lupus erythematosus and a longer duration of use of steroids. Eight of the 10 patients had coexistent coronary artery disease or transient ischaemic attack.
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PMID:Peripheral vascular disease in patients with systemic lupus erythematosus. 154 39

Antiphospholipid antibodies are a relatively heterogeneous mix of immunoglobulins with binding specificities for negatively charged or neutral phospholipids. Currently, the most commonly detected antiphospholipid antibodies include the anticardiolipin antibody, the lupus anticoagulant, and an antibody implicated in false-positive VDRL testing. Recently, a clinical syndrome of vaso-occlusive disorders associated with antiphospholipid antibodies has been identified and may result from immune-mediated disruption of endothelial function. This clinical syndrome encompasses arterial and venous thrombosis, recurrent fetal loss, neurologic dysfunction (eg, migraine, chorea, and encephalopathy), systemic and pulmonary arterial hypertension, and endocardial disease. Although most commonly associated with systemic lupus erythematosus, the antiphospholipid antibody syndrome also has been identified in patients with vaso-occlusive disease without systemic lupus erythematosus. Recently, identification of antiphospholipid antibodies has been facilitated by the development of a more sensitive assay for anticardiolipin antibody. In this article, case histories of three patients with arterial thrombosis and associated anticardiolipin antibodies, including the first associated case of terminal aortic thrombosis, are reviewed and the subject of the antiphospholipid antibody syndrome is discussed.
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PMID:Antiphospholipid antibodies and arterial thrombosis. Case reports and a review of the literature. 155 Apr 84

In a retrospective case control study of 96 obstetrical patients 48 cases had partial thromboplastin time (TTPA) with kaolin over 4 seconds compared with the test group. The control group of 48 women with normal TTPA were also studied. Age, socioeconomic status, weight, family and personal illness history were included. Habitual abortion,neonatal death, and hypertension were recorded. The average TTPA value was 53.6 +or- 7.87 seconds for the case group vs 38.8 =or- 4.9 for the controls which was not statistically significant. No statistical significance was found regarding age, start of menarche, nutritional and socioeconomic status, and blood group. The body weight of the case group was higher with 58.5 kg =or- 14.4 kg (a range of 43.4-81.4 kg). There were 7 cases of thrombophlebitis (14.5%) in the lower extremities in the case group and none in the controls. There were 7 cases of habitual abortion in the case group defined as 3 or more miscarriages before 20 weeks of gestation vs 2 cases in controls. There were 4 cases of neonatal deaths associated with premature delivery in the case groups and none in controls. Acute hypertensive disease associated with pregnancy totaled to 8 cases in the 1st group (16.6%) and 4 cases in controls (8.3%). In both groups there were 2 cases of fetal death. In the case group there was 1 case of chromosomopathy and in the control group 1 case of premature expulsion of placenta. The TTPAs test is used mostly for the initial phase of studying patients suspected of having lupus anticoagulant (LA). LA belongs to abnormalities characterized by the presence of antiphospholipid antibodies. It is often used for diagnosing initial stages of autoimmunity which can frequently occur in thrombotic process, fetal loss, intrauterine growth retardation, and increased hypertensive illness in pregnancy.
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PMID:[Presence of inhibitors of activated partial thromboplastin time (TTPA). Clinical repercussion in obstetric patients]. 155 88

We present a non-controlled prospective study of 10 hypertensive patients with systemic erythematous lupus and nephropathy, treated with captopril exclusively or combined with other drugs, in order to assess its effectivity and potential side effects. Four of these 10 patients had mild hypertension; 3, moderate hypertension and 3, severe hypertension. In 5 of them, arterial pressures was controlled with just captopril; in other 3, we added furosemide and in one patient, we added furosemide and nifedipine. In one case, hypertension was not controlled. Renal function remained stable and proteinuria improved in six patients. Three patients presented reversible agranulocytosis, during or immediately after treatment. One of them was treated two years after with enalapril, without observing hematologic recurrence. We conclude that captopril is useful in treating arterial hypertension associated to lupous nephropathy, but frequent leukocyte counting controls must be done during the first months.
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PMID:[Treatment of arterial hypertension with captopril in lupus nephropathy]. 821 76


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