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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 authors report their comparative experience of the treatment of proliferative lupus glomerulonephritis using prednisone (16 patients) or the indomethacin-hydroxychloroquine association (12 patients). Prednisone in high dosage was associated in this series with 9 deaths and in 6 patients, with renal failure or an extra-renal complication. By contrast, the indomethacin-hydroxychloroquine association proved to be highly effective, without side-effect. In the endocapillary glomerulonephritis group (8 cases) the authors obtained 7 durable remissions (36.3 months on average) and 1 temporary remission of 24 months, with an average length of treatment of 45.8 months. In the extracapillary glomerulonephritis group (4 cases) the authors obtained 1 remission, 2 improvements and I death, with an average length of treatment of 16.8 months. This combination has a highly significant anti-proteinuric and anti-haematuric action, with a constant efficiency on renal function and on the extra-renal signs of lupus. Its effect is less constant on the immunological disorders. Study of iterative renal biopsies confirms this favourable impression. According to these results, the authors propose a provisional scheme of management of proliferative lupus glomerulonephritis.
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PMID:[Lupus nephropathy. Treatment with the indomethacin-hydroxychloroquine combination and comparison with corticoids]. 113 30

A dynamic estimation of the involvement of the complement system in various diseases was obtained by the direct quantitation of breakdown products of C3 and of properdin factor B. The methods used were based, first on the separation of native and fragmented molecules according to their molecular size through a precipitation with polyethylene glycol and, secondly, on an immunochemical quantitation, using specific antisera for the major antigens of C3 and factor B. The sensitivity and the specificity of these methods were demonstrated by activation of complement in vitro with generation of C3 and factor B fragments. A clinical investigation was carried out in 41 patients with systemic lupus erythematosus (SLE), 31 with membranoproliferative glomerulonephritis (MPGN), 26 with other types of glomerulonephritis, and 6 with severe alcoholic cirrhosis of the liver. The following observations were made: (a) an elevated plasma level of C3d fragment of C3 was found in 68% of SLE patients, in 87% of MPGN patients, in 62% of patients with other hypocomplementemic nephritis, and in 15% of those with normocomplementemic nephritis, but in only 33% of patients with liver cirrhosis and very low levels of C3; (b) a significant difference was observed between the levels of C3 obtained with either anti-"native" C3 or anti-C3c sera for immunochemical quantitation, in patients with SLE or MPGN, indicating the presence of "altered" or fragmented C3 in plasma; (c) an elevated plasma level of Ba fragment of properdin factor B was found in 46% of SLE patients, in 67% of MPGN patients, in 50% of patients with other hypocomplementemic nephritis, and in 9% of patients with normocomplementemic nephritis, while the level of properdin factor B was only slightly decreased in these diseases; (d) in SLE and MPGN there was an inverse correlation between the levels of C3d and Ba and the level of C3 in plasma. The level of these fragments was directly correlated with the clinical manifestations of SLE; (e) some patients with a normal C3 level exhibited an elevated plasma concentration of C3 and factor B fragments, suggesting the coexistence of an increased synthesis with a hypercatabolism of complement components. Therefore, the quantitation of complement breakdown products by simple immunochemical methods provides additional information concerning the involvement of complement in disease and new features for the evaluation of the intensity of immune reactions during immune complex diseases.
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PMID:Complement breakdown products in plasma from patients with systemic lupus erythematosus and patients with membranoproliferative or other glomerulonephritis. 114 31

This study demonstrates that in systemic lupus erythematosus (SLE), the presence of immune complexes on the glomerular basement membrane (GEM) does not invariabley result in histological and/or functional lesions of the kidney. Among a group of 29 lupus patients, six subjects were selected for thorough investigation, because their renal function was normal or only slightly altered though they had suffered from SLE for 20 months to 18 years. All patients had antinuclear factor, anti-native-DNA antibody and a low level of complement; 3 had anti-denatured-DNA antibody, 2 had denatured DNA-anti-denatured-DNA circulating complexes and 3 had anti-RNA-protein antibody. Kidney biopsies disclosed either no histological lesion or minimal changes in five of them and diffuse proliferative glomerulonephritis in one. By contrast, using the immunofluorescent technique, granular deposits containing the third component of complement (C3) were found on the GBM of all patients; IgG was present in 5 cases, IgM in 3, fibrinogen in two cases and around the tubules of one. Electron microscopy confirmed the presence of subendothelial and mesangial deposits. Our results also showed a good correlation between the importance of deposits and the presence of denatured DNA-anti-denatured-DNA circulating complexes. From the data obtained in these 6 cases as well as in the 23 other patients of the group, 3 categories of lupus patients could be distinguished with regard to kidney involvement: 1) patients with insignificant histological lesions, no immune deposits and essentially normal function; 2) patients with definite histological lesions, immune deposits and renal insufficiency and 3) patients with few if any histological lesions and little functional impairment contrasting with important immune deposits. The resistance of some patients to the mephrotoxic effects of immune deposits shows that lupus nephritis depends on intricate pathogenic mechanisms and suggests that these are possible antagonized by "protective" factors.
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PMID:Immune complex deposits in systemic lupus erythematosus kidney without histological or functional alterations. 114 88

Properdin deposition has been recognized in glomeruli of patients with acute and chronic nephritis and lupus nephritis, and low serum properdin levels have been found in these disorders. These findings suggest that properdin may be involved in the production of glomerular damage and that low properdin levels may be due to hypercatabolism. The study was designed to examine the metabolism of properdin in normal subjects and to look for an abnormality in five patients with systemic lupus erythematosus with renal involvement and in six patients with membranoproliferative glomerulonephritis or dense deposit disease (MPGN). Highly purified human properdin was prepared by elution from zymosan, followed by DEAE-cellulose and carboxymethyl-Sephadex chromatography, and labeled with 125I by the iodine monochloride method. Parameters of metabolism were determined by monitoring plasma and urinary radioactivity at frequent intervals after the intravenous injection of 1-2 muCi of labeled material. The fractional catabolic rate (FCR) of properdin in normal subjects was found to have a very narrow range of 0.78-1.0,% of the plasma pool per hour (mean 0.95%). In systemic lupus erythematosus, the FCR was regularly elevated with a range of 1.21-2.30% (mean 1.70%). In MPGN, FCR was elevated in three patients (1.22, 1.94, and 2.08%) and within or below the normal range in three (0.78, 1.00, and 1.00%). Properdin levels were reduced in two patients who had the highest FCR's noted in the study. Properdin synthetic rates in normals varied from 4.1 to 14.3 mug/kg per h (mean 9.1) and was not found to be reduced in any patient. Properdin catabolism was found to be normal in a patient deficient in the C3b inactivator. These studies show that properdin is hypercatabolized in patients with renal disease and that decreased properdin levels when they occur in these patients can be entirely explained on the basis of this hypercatabolism.
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PMID:Metabolism of properdin in normal subjects and patients with renal disease. 115 85

The course of systemic lupus erythematosus (SLE) in 34 patients receiving prednisone and immunosuppressive (cytotoxic) drugs is described. 31 patients are in continuous ambulatory care, 27 of them 3 to 17 years. Only two were hospitalized for therapeutic purposes: one patient because of exacerbation of lung tuberculosis; the second patient, who discontinued prednisone therapy despite active SLE, because of severe autoimmune haemolytic anaemia. The retrospective analysis of symptoms demonstrated that only 22 of the 31 patients manifested 4 or more of the prelimievious reports. Two patients with severe lupus glomerulonephritis and azotaemia at the time of initial evaluation did not benefit from combined therapy. Nine patients with mesangial and focal glomerulonephritis have normal kidney function up to 12 years after diagnosis (in two cases kidney function improved after therapy).
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PMID:[Classification and immunosuppressive therapy of systemic lupus erythematosus: long-term follow-up of 31 patients (author's transl)]. 120 30

Direct cutaneous immunofluorescence microscopical examination of uninvolved skin is an important diagnostic test in systemic lupus erythematosus. Its prognostic significance is undetermined. In twenty-four patients there was an increased incidence of leukopenia, hypocomplementaemia, and LE cells in patients with positive skin immunofluorescence. Positive cutaneous immunofluorescence of uninvolved skin was correlated with the most severe forms of lupus renal disease, membranous glomerulonephritis, and diffuse proliferative glomerulonephritis.
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PMID:The significance of a positive cutaneous immunofluorescence test in systemic lupus erythematosus. 120 77

The time course of DNA binding capacity (DNA-bc) was found to correlate with the clinical course in a group of 21 patients who had biopsy-proven lupus glomerulonephritis and were treated with immunosuppressive agents. Eight patients showed a sequential DNA-bc pattern in which a high titre of anti-DNA antibody was present for a prolonged period of time all having an unfavourable clinical course. Persistently high values of DNA-bc preceded by as much as 10 months evidence of renal deterioration obtained by conventional renal function tests. Thirteen patients showed a low titre of anti-DNA antibody throughout most of their course, 12 having a favourable outcome. An initially high value of DNA-bc had no prognostic significance. These results suggest that the persistence of a high titre of anti-DNA antibody in patients with lupus glomerulonephritis is a poor prognostic sign.
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PMID:Prognostic significance of DNA-binding capacity patterns in patients with lupus nephritis. 122 25

Extramembranous glomerulonephritis is an uncommon but distinct pathologic lesion in children. The diagnosis is established by the characteristic light, immunofluorescent, and ultrastructural abnormalities in renal biopsy specimens. This report describes seven of the ten children with this lesion studied in the past 11 years. Emphasis is given to the comparison of four children with idiopathic membranous glomerulonephritis with three others who presented with a nephrotic syndrome but subsequently developed evidence of systemic lupus erythematosus. Two of the latter three children, and three others with SLE and MGN not described in detail, demonstrated deposition of IgA by immunofluorescence along glomerular capillaries. Five of six children with SLE and MGN had microtubular structures in glomerular endothelial cells demonstrable by electron microscopy. These observations suggest that children with MGN require careful and continuing study for evidence of SLE.
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PMID:Extramembranous glomerulonephritis in childhood: relationship to systemic lupus erythematosus. 124 50

Sera from patients with various types of glomerulonephritis (GN) as well as sera from rabbits with acute serum sickness were studied for the presence of circulating immune complexes (IC). The method used is based on the observation that IC inhibit the uptake of IgG aggregates by guinea-pig peritoneal macrophages. Inhibition significantly greater than with normal human sera was found with sera of patients with membranous GN, membranoproliferative GN, focal glomerular sclerosis, minimal change nephrotic syndrome, acute septicaemic glomerular diseases and systemic lupus erythematous. IC were also detected in rabbits with acute serum sickness during the period of immune elimination.
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PMID:Detection of circulating soluble immune complexes in patients with various renal diseases. 124 61

C1q deposits are usually found in association with other complement components and immunoglobulins in proliferative glomerulonephritis and may predominate in systemic lupus erythematosus (SLE). We report the clinical outcome of four patients who developed a nephrotic syndrome associated with C1q nephropathy unrelated to SLE. On presentation the mean urinary protein loss was 6.8 g/24 h (range 4-10), and renal function impaired, mean serum creatinine 201 mumol/l (150-400). Over a mean follow up period of 6.5 years (1.7-19), all four patients improved, three spontaneously and one treated with steroids and cyclosporin, to a current urinary protein loss of 0.3 g/24 h (less than 0.2-0.9) and serum creatinine 98 mumol/l (68-115). C1q nephropathy was confirmed in each biopsy by conventional immunohistology. C1q deposits were demonstrated within the glomerular basement membrane of three biopsies and the mesangium in two samples. One patient had been categorized on light- and electron-microscopy as having mesangiocapillary glomerulonephritis, one membranous glomerulonephritis, one proliferative glomerulonephritis with focal segmental glomerulosclerosis, and one diffuse proliferative glomerulonephritis with both subendothelial and mesangial dense deposits. In view of the expected progressive nature of the underlying renal histopathological appearance, the presence of predominant C1q deposits would appear to be associated with a better clinical outcome.
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PMID:C1q nephropathy: do C1q deposits have any prognostic significance in the nephrotic syndrome? 132 73


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