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)

As systemic lupus erythematosus (SLE) is an uncommon disorder of childhood and is rarely seen among children in developing countries, management is not based on secure criteria and is often a matter of individual preference. In our previous experience, all four children suffering from SLE died soon after diagnosis. Aggressive therapy has been recommended to deal with such problems in the third world. We now report improved management of four more children with SLE seen since 1988 at King Edward VIII Hospital and observed for from 21 to 57 months. All our patients had evidence of renal involvement, with severe disease in two patients confirmed on renal biopsy; three had neurological involvement. The mainstay of treatment was a combination of steroids, azathioprine and chloroquine together with pulse methylprednisolone or cyclophosphamide for severe relapses. Disease activity was closely monitored, using clinical and laboratory criteria. Following therapy, all patients are in a stable condition and in clinical remission; one has mild renal impairment. These early results suggest that the judicious use of a few drugs, together with regular and meticulous follow-up, can greatly improve the prognosis of childhood SLE, even in third world countries.
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PMID:Improved outcome of systemic lupus erythematosus among children in Durban, South Africa. 752 26

Although several clinical and morphological changes observed in overt systemic lupus erythematosus have been associated with the presence of antiphospholipid antibodies (aPL), the relation between these antibodies and lupus nephropathy (LN) is not clear. Twenty-three patients with biopsyproved LN were retrospectively studied (average age 28.5 +/- 12.3 years, all women) in order to investigate the relationship between the presence of aPL and clinical and immunobiological data. The average follow-up period was 55 +/- 42 months. The presence of aPL (IgG and IgM) was detected at least once in all patients by ELISA and/or lupus anticoagulant (kaolin time). Seven patients (30.4%) were aPL+, and the remainder aPL-. We did not find differences related to age, period of follow-up, blood pressure and livedo reticularis. However the prevalence of thrombosis, strokes and hemolysis was slightly higher in the aPL+ patients. The levels of antinuclear antibodies or anti-DNA antibodies, immunoglobulins and complement serum levels (C3, C4) were also similar in both groups. In the aPL+ group, proteinuria was significantly higher than in aPL- cases (2.21 +/- 1.5 and 0.91 +/- 1.07 g/24 h, respectively; p = 0.029). The renal histological pattern in both series was similar. However, microthrombosis in the glomerular capillary lumens was more frequent in the aPL+ group. The evolution of renal function was less favorable in aPL+ patients when compared with aPL- patients. We conclude that the presence of aPL in patients with LN is associated with several characteristics of renal impairment which may contribute to its evolution.
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PMID:The role of antiphospholipid antibodies in lupus nephropathy. 853 46

We have measured the urinary excretion of total protein, albumin and retinol binding protein (RBP) in random urine specimens obtained from 40 female patients with systemic lupus erythematosus (SLE). Thirty-three of these patients had no clinical evidence of any renal impairment (non-renal SLE); seven had overt renal disease (renal SLE). RBP:creatinine ratios were significantly higher in non-renal SLE patients compared with female controls (P = 0.002). There was no significant difference between urine total protein concentrations, albumin:creatinine or total protein:creatinine ratios in non-renal SLE patients when compared with controls, despite approximately 20% of these patients having elevated excretion of total protein or albumin. All seven renal SLE patients had elevated albumin:creatinine ratios but only four of them had an increased RBP:creatinine ratio. Of 29 non-renal SLE patients who had urinary total protein concentrations below 0.2 g/L, (i.e. approximating to a negative protein dipstick), 14 had increases in either albumin or RBP:creatinine ratios. Only two patients had increases in both. In the absence of clinical evidence of renal disease, increases in urinary albumin or RBP excretion could indicate subclinical nephropathy and measurements may have a role in the early diagnosis and subsequent monitoring of renal disease in SLE.
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PMID:Urinary excretion of albumin and retinol binding protein in systemic lupus erythematosus. 936 6

A 49 year-old man was admitted for edema and renal impairment due to SLE. Since he did not improve with predonisolone and methylprednisolone pulse therapy, cyclophosphamide pulse therapy (300 mg div.) was administered. The patient subsequently developed a fever, dyspnea and cough, and interstitial regions of the lungs exhibited shadows on X-ray and CT. The patient also suffered hypoxemia and poor lung function. Since several culture tests and viral antibody tests were negative for infection, antibiotics were not effective, and TBLB indicated interstitial pneumonia, which we speculated was induced by cyclophosphamide. However, this was such a severe case of interstitial pneumonia that it could not be cured merely by discontinuing the cyclophosphamide, but it did improve immediately after starting methylprednisolone pulse therapy. The incidence of cyclophosphamide-induced interstitial pneumonia is very low, but the mortality rate is high. Since cyclophosphamide pulse therapy is often used to treat SLE, attention should be focused on the incidence of interstitial pneumonia.
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PMID:[SLE with interstitial pneumonia during cyclophosphamide pulse therapy]. 980 18

Within a 6-year period from January 1991 to December 1996, 19 patients with Salmonella choleraesuis bacteremia were enrolled for clinical and microbiological analysis. Young children, the elderly and patients with hematological malignancy (36.8%), liver cirrhosis (26.3%), systemic lupus erythematosus (10.5%), chronic renal impairment (10.5%), and peptic ulcer (10.5%) were at high risk of this infection. The ratio of male to female was 3:1. Three cases (15.8%) were nosocomially acquired. Fever (89.5%), chills (57.9%) and anorexia (52.6%) were the most common clinical manifestations. Seven patients (36.8%) presented no gastrointestinal manifestations. Normal white blood cell count was noted in seven patients (36.8%), and neutropenia caused by underlying diseases or severe infection was found in six cases (31.6%). Various types of metastatic focal infections were found, such as septic arthritis, cutaneous infection, spontaneous bacterial peritonitis, and pneumonia. The severe immunocompromised status of patients and the high virulence of this pathogen may contribute to the high case fatality rate (21%). Higher resistance rate to commonly used antimicrobial agents was noted in ampicillin (94.7%), chloramphenicol (89.5%), and TMP/SMZ (63.8%). All strains of S. choleraesuis were susceptible to third-generation cephalosporins and fluoroquinolones. Generally, S. choleraesuis bacteremia should be taken into account in the differential diagnosis of sepsis in immunocompromised patients, even without gastrointestinal manifestations. The third-generation cephalosporins and fluoroquinolones may be the first choice for treatment of this invasive infections.
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PMID:Salmonella choleraesuis bacteremia in southern Taiwan. 1033 Jul 99

The aims were to study the gender differences in clinical manifestations, disease course and organ damage in systemic lupus erythematosus (SLE). Clinical manifestations, autoantibody profile, relapses and damage scores were obtained from 51 Chinese males with SLE and compared with 201 consecutive female SLE controls. Fifty-one males were identified among 630 SLE patients who attended our clinics, giving a male prevalence of 8% and a female to male ratio of 11.4-1. Both the male SLE patients and the female controls had similar age and SLEDAI score at disease onset. Male SLE patients had less alopecia (P = 0.03), Raynaud's phenomenon (P = 0.01) and anti-Ro (P = 0.049) during the course of the disease but none of the differences were statistically significant after correction for multiple observations. The prevalence of major organ involvement in either sex was not different. Both groups of patients had a comparable mean duration of follow-up (104 vs. 102 months, P = 0.87). Males had a significantly lower rate of relapses (total No. of flares/patient-year: 0.23 in men vs. 0.33 in women, P = 0.04), but the frequency of severe flares (No. of severe flares/patient-year in men 0.08 vs. 0.12 in women, P = 0.16) was not significantly different from the females. Male patients with positive anti-Ro had significantly less overall flares than their female counterparts who were anti-Ro positive (0.16 vs. 0.34, P = 0.006). However, the use of immunosuppressive agents for disease control in patients of both sexes was similar. 22 (43%) of the males and 78 (39%) of the females had organ damage. A higher percentage of male patients had impairment of renal function (P = 0.006) but the proportion of patients who required dialysis was not different (4% in men vs. 2% in females. P = 0.92). There was also a trend of more cardiovascular damage in the males but the difference was not statistically significant (P = 0.09). The mean SLICC/ACR scores were not significantly higher in the males than the females (0.71 vs. 0.60, P = 0.47). Males tend to differ from females in clinical manifestations, immunological profile and disease course in SLE. However, there was no gender difference in the involvement of major organs/systems. Males had less overall disease flares than the females but the rate of severe flares was not significantly lower. For patients who were anti-Ro positive, males had significantly less total number of flares/patient-year than their female counterparts. More renal impairment and cardiovascular damage was present in our male lupus patients but the overall damage scores were not significantly higher.
Lupus 1999
PMID:Clinical characteristics and outcome of southern Chinese males with systemic lupus erythematosus. 1034 11

Renal diseases of mixed connective tissue disease (MCTD) are not unusual. Although most of them are SLE-like renal impairment with immune complex deposits, systemic sclerosis- (SSc) like renal impairments with intimal thickening of interlobular arteries or arterioles are also encountered. Several cases of SSc complicated with MPO-ANCA-related necrotizing glomerulonephritis (nGN) are reported. Here we report a case which developed MPO-ANCA-related nGN 16 years after the diagnosis of MCTD. She exhibited pauci-immune focal nGN and significantly high titer of MPO-ANCA. She was successfully treated with prednisolone and cyclophosphamide. We believe this is the first case in which MPO-ANCA-related nGN was demonstrated in a patient with MCTD.
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PMID:A case of mixed connective tissue disease (MCTD) complicated with MPO-ANCA-related necrotizing glomerulonephritis. 1096 96

Individual case reports have documented nephrotoxicity of intravenous immunoglobulin (IVIG) preparations, but the true incidence of renal dysfunction is unknown and many data sheets do not include renal impairment as a side-effect of these preparations. We determined the incidence of renal impairment in an unselected cohort of patients receiving two different preparations of IVIG over 20 months, administering 287 courses of IVIG to 119 patients for a variety of indications, including thrombocytopenia, systemic lupus erythematosis, neuropathy, Guillain-Barre syndrome and infections. Two different preparations of IVIG were used, Vigam (BPL) and Sandoglobulin (Novartis), which differ in the concentration of sucrose added as a stabilizer. Eight patients showed deterioration in renal function (6.7%), and in two, no renal recovery occurred (1. 7%). There were no significant differences in the patient characteristics or dose or preparation of IVIG administered to those patients with or without changes in serum creatinine. There was no association between the amount of sucrose in the IVIG and development of renal failure. IVIG (regardless of the sucrose content) is associated with renal impairment which may be irreversible, with a maximum incidence of 6.7%. All patients should have their renal function monitored during the use of IVIG.
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PMID:Nephrotoxicity of intravenous immunoglobulin. 1107 32

Cyclosporin is a potent immunosuppressive drug and is frequently used in the therapy of autoimmune diseases, including systemic lupus erythematosus (SLE). Few large studies have been performed using this drug in SLE patients. However, small uncontrolled studies of patients with SLE have shown favourable results with a significant improvement in disease activity, a fall in anti ds DNA titres and proteinuria and an improvement in complement levels, leucopaenia and thrombocytopaenia. Interestingly, a consistent reduction in corticosteroid dosage often by as much as 50% is seen. Toxicity, especially with hypertension and renal impairment, occurs but usually reverses on dose reduction or the addition of an anti-hypertensive agent and is minimised by adherence to the strict monitoring guidelines. Large multi-centre randomised-controlled trials of the use of cyclosporin in SLE patients are underway and the results are eagerly awaited.
Lupus 2001
PMID:The treatment of lupus with cyclosporin A. 1131 47

Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) can be associated with significant morbidity in children and adolescents. Renal involvement in SLE appears to be more severe and more frequent in the pediatric age group, with the major predictors for poor outcome being the severity of histopathologic lesions, severity of renal impairment at diagnosis, and hypertension. In addition to currently recognized cardiovascular and pulmonary involvement, accelerated atherosclerosis is of increasing concern in young individuals with SLE, because of both disease effects and medication usage. Neuropsychiatric SLE seen in childhood ranges from subtle cognitive dysfunction to severe central nervous system involvement; however, there is controversy over the value of different diagnostic studies. APS in children may be associated with SLE, idiopathic, or associated with viral infections. Systemic anticoagulation is recommended for patients with thrombotic events, but long-term management has not been well studied in children.
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PMID:Systemic lupus erythematosus and antiphospholipid syndrome in children and adolescents. 1160 98


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