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)

A retrospective analysis of the long-term outcome of patients with membranous lupus nephropathy (MLN) was conducted. One hundred Chinese patients, 90 females and 10 males with a mean age of 32+/-9 years, with systemic lupus erythematosus and biopsy-proven MLN (ISN/RPS2003 classification criteria) were enrolled in this study. The patient and renal survivals were estimated by the Kaplan-Meier method and the risk factors associated with end-stage renal failure (ESRF) were assessed by the Cox proportional hazards regression analysis. The mean follow-up of all patients was 77.6+/-56 months. During follow-up, two patients died. Patient survival at 5 and 10 years was 98%. Renal survival at 5 and 10 years was 96.1% and 92.7%, respectively. Severe tubular-intersticial lesion (HR 66.514), nephrotic range proteinuria (HR 19.159) and refractoriness to treatments (HR 9.834) were independent risk factors for developing ESRF. Three of the six patients with ESRF had severe tubular-interstitial lesions on initial biopsy. Twenty-one patients underwent a repeat biopsy after 33months' (median time) follow-up, eight (38.1%) of these (class V superimposed class IV in 5, class V superimposed class III in 2 and class VI in 1) had transformed and three (37.5%) of them progressed to ESRF. Complications included infection (13%), thrombosis (3%), avascular necrosis (3%), diabetes mellitus (4%) and skin cancer (1%). The rate of patient and renal survival was high in this group of patients with MLN.
Lupus 2008 Jan
PMID:Long-term outcome of Chinese patients with membranous lupus nephropathy. 1808 85

A 29-year-old male presenting nephrotic syndrome and facial skin erythema was admitted to our hospital in September of 2000. We diagnosed him as having systemic lupus erythematosus (SLE) accompanied by lupus nephritis (WHO class V). The disease activity had decreased after treatment with methylprednisolone (m-PSL) pulse therapy, which was followed by oral PSL. Thereafter, when tapering the dosage from 60 to 30 mg/day, the lupus nephritis flared up and he was re-hospitalized in February of 2001. After successful retreatment with m-PSL pulse therapy followed by the tapering of the dosage from 60 to 30 mg/day, we used mizoribine (MZR) as a combination therapy. The lupus nephritis flared up again after tapering down to 17.5 mg/day of PSL. Then, we changed the MZR dosage from 150 mg/day in three divided daily doses to 200 mg/day in two divided daily doses. This modification increased the peak blood concentration (Cmax) of MZR from 0.63 to 1.55 microg/ml. At present, we have been able to successfully taper the dosage to 7.5 mg/day of oral PSL and the patient has achieved a state of remission without any side effects. Monitoring of the serum concentration of MZR is thus considered to be important for achieving effective therapy of SLE, especially for steroid-resistant lupus nephritis. If the serum concentration of MZR does not reach an effective level, then the dosage of MZR should be adjusted appropriately in order to maintain an adequate serum concentration of MZR.
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PMID:A case of lupus nephritis improved after appropriately adjusting the dosage of mizoribine. 1815 69

Lupus nephritis is a common complication of systemic lupus erythematosus (SLE). Early recognition of lupus nephritis requires routine serum creatinine determination, urinalysis and urinary microscopy. Since mild urinary abnormalities such as leucocyturia or proteinuria can be associated with severe lupus nephritis, a renal biopsy is usually indicated in patients with SLE and urinary abnormalities. A renal biopsy is required to determine the class of lupus nephritis which is based on histopathological criteria which have recently been revised. Aggressive immunosuppressive therapy is indicated in diffuse proliferative lupus nephritis. In class III or class V the treatment indication depends on additional prognostic criteria. Intravenous cyclophosphamide is still used but doses and intervals have been modified based on large clinical trials. Mycopheno-late may establish as an alternative for cyclophosphamide in the induction phase, but the data of the transcontinental multicenter Aspreva Lupus Management Study (ALMS) trial have not yet been published in detail. Controlled clinical trials support the use of azathioprine and mycophenolate for maintaining remission of lupus nephritis, and cyclophosphamide is no longer used in that phase. Additional control of cardiovascular risk factors and combined angiotensin and angiotensin receptor blockade are mandatory for all proteinuric SLE patients. Novel treatment options are ahead of us based on the molecular mechanisms of SLE and lupus nephritis, but as evidence from controlled clincial trials is still lacking they are not yet approved for broad clinical use. However, the treatment options for severe lupus nephritis have been improved and are likely to further improve in the near future.
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PMID:Lupus nephritis. 1829 86

Recently a role of the upregulation ofcyclooxygenase isoforms in renal injury and modulation the severity of the inflammatory reactions is suggested. Cyclooxygenase exists as two isoforms COX-1 and COX-2 which are poorly understood with regard to their roles in renal function. Thereby, the present study was undertaken to ascertain the immunoexpression of cyclooxygenase-1 (COX-1) and cyclooxygenase-2 (COX-2) in lupus (LMGN) and nonlupus (NLMGN) membranous glomerulopathy and to examine the possible relationship between this immunoexpresion and inflammatory infiltrates. Eleven renal biopsy specimens from patients with class V lupus glomerulopathy and 16 from patients with primary (nonlupus) membranous glomerulopathy were examined by percutaneous renal biopsy. As a control 10 biopsy specimens of the kidneys removed because of trauma were used. In each specimen staining intensity of COX-1 and COX-2 in glomeruli, tubuli, arterioles and interstitial cells were recorded semiquantitatively whereas CD68+ cells, CD3+ cells and CD20+ cells were assessed quantitatively using computer image analysis system. Our study revealed that the mean scores of COX-1 immunoexpression did not differ significantly in all groups investigated whereas immunoexpression of COX-2 in LMGN was significantly stronger as compared with both NLMGN and controls. Moreover, in LMGN a significant positive relationship was noted between COX-2 immunoexpression and CD 68+ cells. In NLMGN and controls the correlations between COX-2 immunoexpression and CD 68+ cells were positive, but they have not reached statistical significance. In conclusion, our findings point that glomerular inflammation in lupus and non-lupus membranous glomerulopathy have different signalling pathways and suggest that in lupus nephritis COX-2 and monocytes/ macrophages but not COX-1 isoform are involved in the inflammatory process.
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PMID:Analysis of renal immunoexpression of cyclooxygenase-1 and cyclooxygenase-2 in lupus and nonlupus membranous glomerulopathy. 1845 55

We describe a 24-year-old woman with a distinctive glomerular lesion. She presented with nephrotic syndrome and the diagnosis of systemic lupus erythematosus was made on the basis of laboratory and clinical findings. Renal biopsy showed a bubbling appearance of the glomerular capillary wall indicating lupus nephritis class V. On an electron microscopy, the glomerular basement membrane (GBM) was irregularly thickened and contained abundant vesicular and microtubular bodies. In addition, there were many epithelial foot processes infolding into the GBM. A few small deposits were observed beneath the foot processes and around the vesicular and microtubular bodies. Although the clinicopathological significance of podocytic infolding has not been fully elucidated, it may be a novel morphological entity in the glomerulonephritides.
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PMID:Lupus nephritis with podocytic infolding and intramembranous microstructures. 1883 65

Glomerular epithelial cell (podocyte) injury is characterized by foot process retraction, slit diaphragm reorganization, and degradation of podocyte-specific proteins. However, the mechanisms underlying podocyte injury are largely unknown. The ubiquitin C-terminal hydrolase-L1 (UCH-L1) is a key modulator of ubiquitin modification in neurons. Like neurons, UCH-L1 expression was associated with an undifferentiated status in cultured human podocytes, whereas differentiation and arborization decreased UCH-L1 and monoUb expression. Inhibition of UCH-L1 induced time and concentration-dependent process formation with alpha-actinin-4 distribution to the cell membrane and processes. An immunohistochemical approach was used to evaluate whether UCH-L1 expression was associated with podocyte injury in 15 different human glomerular diseases. Whereas normal kidneys expressed no UCH-L1 and little ubiquitin, a subset of human glomerulopathies associated with podocyte foot process effacement (membranous nephropathy, SLE class V, FSGS) de novo expressed UCH-L1 in podocyte cell bodies, nuclei, and processes. Interestingly, UCH-L1 expression correlated with podocyte ubiquitin content and internalization of the podocyte-specific proteins nephrin and alpha-actinin-4. In contrast, minimal change glomerulonephritis, a reversible disease, demonstrated minimal UCH-L1 and ubiquitin expression with intact alpha-actinin-4 but internalized nephrin. Glomerular kidney diseases typically not associated with foot process effacement (SLE class IV, ANCA+ necrotizing GN, amyloidosis, IgA nephritis) expressed intermediate to no UCH-L1 and ubiquitin. These studies show a role for UCH-L1 and ubiquitin modification in podocyte differentiation and injury.
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PMID:A new role for the neuronal ubiquitin C-terminal hydrolase-L1 (UCH-L1) in podocyte process formation and podocyte injury in human glomerulopathies. 1898 19

This study aimed to analyze the clinicopathological characteristics of familial SLE patients with lupus nephritis (LN). A total of 136 Chinese patients with lupus nephritis diagnosed by renal biopsy, including 34 familial patients and 102 sporadic cases, were recruited. Their demographic information, age of onset, disease duration, clinical features, laboratory data and histological manifestations were compared. The first-degree relatives (17 sibling cases and eight parent-offspring cases) of familial SLE patients were primarily affected. The prevalence of fever, rash and arthritis in familial subjects was higher than that in sporadic subjects. Familial patients had lower platelet counts and higher low-density lipoprotein. In familial patients, class V lupus nephritis was less common. After adjusted with the Benjamini and Hochberg procedure, however, fever was the only feature occurring significantly and more frequently in familial patients (58.8% vs 26.5%, P = 0.001). Moreover, SLEDAI and other clinicopathological features did not differ significantly between the two groups. Multivariate analysis showed that a higher prevalence of fever was an independent predictor of familial SLE. Most clinicopathological features in familial SLE patients were not significantly different from those in sporadic patients; the severity of LN in familial SLE patients was similar to that of sporadic cases. Thus, familial SLE may not be a different clinical entity.
Lupus 2009 Mar
PMID:Clinicopathological characteristics of familial SLE patients with lupus nephritis. 1921 63

We report the clinical profile, treatment and outcome of systemic lupus erythematosus in 70 patients between the age of 4-15 years. Fever (94.2%), arthritis (65.7%) and malar rash (57.1%) were the chief extra-renal manifestations. The ESR was raised in 98.5% patients, anemia was seen in 60% and direct Coombs test was positive in 58.3%. Antinuclear antibody was positive in all; anti-double stranded DNA antibody and low C3 levels were seen in 77.1% and 80%, respectively. Renal involvement was noted in 77.1% and included proteinuria (53%), hematuria (42.8%), hypertension (18.5% and elevated serum creatinine (8.6%). Renal histology showed class I nephritis in 3.7%, class II in 44.4%, class III in 4.3%, class IV in 44.4% and class V in 1.8%. On follow up 18.8 months later, 70% patients were in remission, 7.5% had active disease and 7.5% died. The characteristics of childhood lupus erythematosus were similar to those previously reported. The outcome was favorable in most cases.
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PMID:Clinical features and outcome of systemic lupus erythematosus. 1921 81

We report the clinicopathological features, treatment and outcome of 54 Indian children (14 boys) with biopsy-proven lupus nephritis followed over a 10-year period. The mean age (SD) at onset of disease was 9.6 +/- 2.6 (range 2.5-14.4) years. Twenty-six (48.1%) patients had class IV nephritis, 7 (13.0%) had class V, whereas class I, II and III nephritis were present in 3 (5.6%), 10 (18.5%) and 6 (11.1%) patients, respectively. Hypertension, haematuria and nephrotic range proteinuria were present in 30 (55.6%), 31 (57.4%) and 28 (51.8%) patients, respectively. Compared with all the other classes combined, there were more boys among patients with class IV nephritis, and hypertension, haematuria, nephrotic syndrome and decreased glomerular filtration rate at presentation were more common. The mean duration of follow-up was 3.1 +/- 2.9 years (median 2.5, range 0.2-10.3 years). Of the 39 patients who were followed-up for at least 1 year, 33 (84.6%) were in complete or partial remission, whereas six (15.4%) had no response to therapy. The incidence of serious infection was 1.5 episodes per 10 patient-years. Nine patients died, of whom four had serious infections or septicaemia, and three developed end-stage renal failure (ESRF). The patient survival rate at 3 years and at last follow-up visit was 88% and 83.3%, respectively, whereas the renal survival rates (without ESRF) were 92% and 94.4% respectively. Cox regression analysis showed no relation of gender, age of onset, presence of hypertension, haematuria and proteinuria, estimated glomerular filtration rate, renal histology and response to therapy to the outcome of death or ESRF. We found lower patient survival rate as compared with data from the developed countries but similar to that seen in developing countries. Serious infections were an important cause of mortality besides renal failure.
Lupus 2009 Apr
PMID:Outcome of lupus nephritis in Indian children. 1927 3

Class V lupus nephritis (LN) occurs in one-fifth of biopsy-proven cases of systemic lupus erythematosus. To study the effectiveness of treatments in this group of patients, we pooled analysis of two large randomized controlled multicenter trials of patients with diverse ethnic and racial background who had pure class V disease. These patients received mycophenolate mofetil (MMF) or intravenous cyclophosphamide (IVC) as induction therapy for 24 weeks, with percentage change in proteinuria and serum creatinine as end points. Weighted mean differences, pooled odds ratios, and confidence intervals were calculated by using a random-effects model. A total of 84 patients with class V disease were divided into equal groups, each group had comparable entry variables but one received MMF and one received IVC. Within these groups, 33 patients on MMF and 32 patients on IVC completed 24 weeks of treatment. There were no differences between the groups in mean values for the measured end points. Similarly, no difference was found regarding the number of patients who did not complete the study or who died. In patients with nephrotic syndrome, no difference was noted between those treated with MMF and IVC regarding partial remission or change in urine protein. Hence we found that the response to MMF as induction treatment of patients with class V LN appears to be no different from that to IVC.
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PMID:Mycophenolate mofetil and intravenous cyclophosphamide are similar as induction therapy for class V lupus nephritis. 1989 Feb 71


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