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 57-year-old man with monoclonal gamma-globulinemia was admitted because of edema and proteinuria. A renal biopsy specimen showed lobular glomerulonephritis associated with deposition of material that was positive for IgG, C3, C1q, fibrin, kappa light chain, and lambda light chain but was not stained by Congo red. Glomeruli showed massive electron-dense deposits with two kinds of unusual, highly organized crystalline structures in the mesangial matrix and peripheral capillary loops. Clinically, the patient had nephrotic syndrome, microscopic hematuria, and hypertension. No Bence-Jones protein or cryoglobulin was found in the urine or serum. Immunoelectrophoresis of blood and urine revealed increased IgG-lambda paraprotein, but no free light chains were found. This case was not associated with amyloidosis, systemic lupus erythematosus, light chain deposition disease, cryoglobulinemia, or multiple myeloma. Immunotactoid glomerulopathy was diagnosed. Treatment with oral prednisone was effective for the management of nephrotic syndrome and renal dysfunction. Glomerular deposition of two kinds of microtubular structure in immunotactoid glomerulopathy has rarely been reported.
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PMID:A case of immunotactoid glomerulopathy with unusual microtubular deposits. 961 98

There is little data on the spectrum of renal diseases in the United Arab Emirates. A renal diseases registry has been set up in an attempt to address this issue nationwide, and we report here the first outcome of this endeavor, a retrospective histopathologic analysis of 490 native kidney biopsies performed on adult patients presenting to four hospitals in the Emirate of Abu Dhabi from 1978 to June 1996. The most common indication for a biopsy was the nephrotic syndrome (54.0%), followed by asymptomatic urinary abnormalities (29.7%), and chronic renal failure (12.7%). Primary glomerular disease accounted for 77.1% of all biopsies. Chronic proliferative glomerulonephritis as a group was the predominant pathology (36.2%), followed by idiopathic membranous glomerulopathy (20.1%), focal segmental glomerulosclerosis (18.3%), minimal change nephropathy (18.3%), and IgA nephropathy (6.3%). Of the patients with secondary kidney diseases, 33 (40.7%) had systemic lupus erythematosis, 27 (33.3%) amyloidosis, 14 interstitial nephropathy, and seven diabetic nephropathy. Kidney biopsies of 187 patients with primary glomerular disease who presented with the nephrotic syndrome were analyzed. In this group idiopathic membranous glomerulopathy, proliferative glomerulonephritis, and minimal change glomerulopathy was found in almost equal proportions (28.3%, 26.6%, 26.2%) with focal segmental glomerulosclerosis (15.4%) accounting for the bulk of the remainder. Though the overall results of this analysis do not show any major differences in the spectrum of primary glomerulopathies in the United Arab Emirates compared with other countries, a slight tendency towards a higher frequency of focal segmental glomerulosclerosis among patients indigenous to the Arabian Peninsular (20.4%) deserves further evaluation.
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PMID:Analysis of 490 kidney biopsies: data from the United Arab Emirates Renal Diseases Registry. 965 Jan 23

We report a case of immunotactoid glomerulopathy with severe hypocomplementemia. The patient was a 47-year-old woman who presented with pitting edema, proteinuria, and hypertension. Serological testings were negative or within normal limits except for hypocomplementemia. There were no findings of hematopoietic diseases, cryoglobulinemia, and systemic lupus erythematosus. The renal biopsy specimen showed membranoproliferative glomerulonephritis with numerous periodic acid-Schiff (PAS)-positive deposits. Under electron microscopy, however, microtubular structure was shown in the mesangial matrix and the subendothelial and subepithelial spaces of the peripheral capillary loops. These histological features were compatible with those of immunotactoid glomerulopathy. Although conventional oral steroid therapy failed to have an effect on proteinuria and hypocomplementemia over 3 months, steroid pulse therapy brought dramatic relief: complete remission of proteinuria and normalization of hypocomplementemia. These findings suggest that intensive immunosuppressive therapy may cure a kind of immunotactoid glomerulopathy with hypocomplementemia.
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PMID:Successful treatment with steroid pulse therapy in a case of immunotactoid glomerulopathy with hypocomplementemia. 1007 87

Subepithelial deposits are a common feature of idiopathic membranous glomerulonephritis (MGN) and lupus membranous glomerulopathy (LMGN). We investigated the spatial arrangement of immunoglobulin G (IgG) and C3c fraction of complement (C3c) in the immune deposits of MGN and LMGN with confocal laser scanning microscopy to correlate specific patterns of IgG-C3 interactions with different diseases. Ten patients with MGN and 8 patients with LMGN (World Health Organization class VB) were selected. A determination of the spatial arrangement of the two fluorochromes and the glomerular area occupied by each fluorochrome was performed for each case. Our results showed MGN specimens have an orderly distribution of IgG and C3c, with each deposit showing an outer ring of sole IgG. IgG was always more abundant than C3c (1,619 +/- 271 v 790 +/- 105 micrometer(2), P = 0.002). In LMGN, IgG and C3c were haphazardly arranged, with deposits made of C3c only and an outer ring of IgG only rarely present. Also, the relative amounts of the two antigens were variable, and two groups could be identified (group 1: IgG, 5,515 +/- 1,179 micrometer(2) v C3c, 4,810 +/- 1,174 micrometer(2); P = 0.02; group 2: IgG, 3,358 +/- 658 micrometer(2) v C3c, 4,047 +/- 740 micrometer(2); P = 0.03). Our data show that diffuse IgG capping of the subepithelial immune deposits is diagnostic of MGN. The absence of an orderly three-dimensional arrangement in LMGN deposits (ie, outer ring of IgG) is likely to render active complement components more readily available to inflammatory activities.
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PMID:Spatial arrangement of subepithelial deposits in lupus and nonlupus membranous nephropathy. 1040 Oct 20

Three patients are described who presented with a glomerulopathy suggestive of lupus nephritis in the absence of other clinical and biological evidence of systemic lupus erythematosus (SLE). Renal biopsies showed a "full-house" immunofluorescence pattern and two patients also had cytoplasmic tubuloreticular inclusions by electron microscopy. All these patients developed antinuclear and anti-double-stranded DNA antibodies 3, 5, and 10 years after their original presentation. Subsequently, 1 patient also developed clinical symptoms of lupus. Reviewing all renal biopsies performed in our department, we found 14 additional patients who presented with a "full-house" immunofluorescence glomerulonephritis in the absence of other features of SLE. After a mean follow-up of 5.8 years, these patients have not developed serological or clinical evidence of SLE. We conclude that a "full-house" glomerulopathy in children may be the first symptom of SLE, especially when cytoplasmic tubuloreticular inclusions are detected. The appearance of other clinical and biological symptoms may be delayed by several years.
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PMID:Delayed onset of systemic lupus erythematosus in patients with "full-house" nephropathy. 1050 27

The purpose of our study was to compare the incidence of peritonitis between continuous ambulatory peritoneal dialysis (CAPD) treatment (Group I) and automated peritoneal dialysis (APD) treatment (Group II) taking into account the same population. We compared 20 patients with a follow-up of 215 patient-months on CAPD and 252 patient-months on APD. Demographic data, diagnosis, peritoneal equilibration test (PET) results, adequacy, and peritonitis rate were analyzed. Diagnoses included glomerulopathy 35%, autosomal dominant polycystic kidney disease (ADPKD) 20%, Type II diabetes 10%, systemic lupus erythematosus 5%, interstitial nephritis 5%, nephrolitiasis 5%, and unknown 20%. PET results showed that the group consisted of 30% high transporters, 45% high-average transporters, and 25% low-average transporters. Kt/V for Group I was 1.3 +/- 0.3, and for Group II, 1.83 +/- 0.48. Creatinine clearance for Group I was 43.64 +/- 7.31 L/week/1.73 m2, and for Group II, 52.42 +/- 13.47 L/week/1.73 m2. Group I presented a peritonitis rate of 8.3 episodes/patient-month, and Group II presented a rate of 18.9 episodes/patient-month. Gram-positive organisms were responsible for 49.8% of episodes of peritonitis in Group I (S. aureus 26.6%, S. epidermidis 16.6%, others 10%) and 83% of peritonitis episodes in Group II (S. epidermidis 46.6%, S. aureus 20%). Gram-negative organisms were responsible for 16.5% of episodes of peritonitis in Group I. No gram-negative peritonitis was seen in Group II. APD patients developed two cases of candida peritonitis. Our preliminary results show that Group II exhibited a decrease in peritonitis rate while achieving better adequacy. In CAPD and APD peritonitis, gram-positive organisms predominated. In APD, we observed an increase in S. epidermidis incidence. No gram-negative organisms were observed in APD. It seems that APD is a safer treatment owing to the lower peritonitis incidence.
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PMID:Comparing peritonitis in continuous ambulatory peritoneal dialysis patients versus automated peritoneal dialysis patients. 1068

A case of mesangioproliferative glomerulonephritis in a 55-year-old woman with selective IgA deficiency and serum antinuclear antibodies who presented with nephrotic syndrome is described. The patient did not have clinical or laboratory features of systemic lupus erythematosus (SLE) other than antinuclear antibodies. Histology of the patient's renal biopsy revealed a mesangioproliferative glomerulonephritis and direct immunofluorescence showed that paramesangial deposits contained predominant IgM with lesser IgG, C3 and C1q. These findings are identical to those previously described in a form of glomerulonephritis associated with IgA deficiency and would be atypical for lupus nephritis. Glomerulonephritis is not a well recognized complication of IgA deficiency, though it has been rarely reported in the literature. This case provides further evidence that IgA deficiency is associated with a unique immune complex-mediated glomerulopathy with characteristic immunopathological and ultrastructural features. It is the first reported case to present with nephrotic syndrome.
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PMID:Nephrotic syndrome in a patient with IgA deficiency-associated mesangioproliferative glomerulonephritis. 1074 Aug 9

A prominent feature of lupus glomerulonephritis is extracellular, predominantly homogeneous electron dense deposits. Fingerprint-like deposits have been reported in 6 to 10% of cases. On electron microscopy, we studied the frequency and characteristics of organised deposits in 227 kidney tissue samples obtained by biopsy in 185 patients with systemic lupus erythematosus (SLE). Fingerprint forms of deposits were demonstrated in 34 biopsies of 32 patients (17.3%). In the control group of 626 kidney biopsies of patients with primary renal and systemic diseases other than SLE, no fingerprint deposits were found. In 227 kidney biopsy samples, fingerprint deposits were found to be associated with mesangial (8.8%), mesangial-subendothelial (3.8%), subepithelial (28.6%), mesangial-subepithelial (11.1%) and mesangial-transmembranous (19.4%) glomerular deposit distribution patterns. They were demonstrated more often at different locations along the peripheral capillary glomerular basal membrane (77.4%) than within the mesangial matrix (43.7%). In extraglomerular locations, fingerprint deposits were present in the interstitium in 8.8%, along the tubular in 23.5% and peritubular capillary basal membrane in 20.5%, in the wall of the arterioles in 64.7% and in the juxtaglomerular apparatus in 18.2% of biopsies. Organised fingerprint deposits consisted of semicircular dark and light lines, each with a diameter of about 10 to 15 nm. Unilaterally, spiky processes at a periodicity of 10 to 15 nm were seen. Among 14 of 185 SLE patients with cryoglobulinemia, fingerprint deposits were demonstrated in only 2 patients. We conclude that fingerprint deposits are characteristic, diagnostically relevant for SLE and represent morphologically a homogeneous group of organised deposits unrelated to cryoglobulins. In 3 SLE patients, 20 to 100 nm tubules and in 2 SLE patients, 10 and 18 nm Congo red negative fibrils were found. By their morphology and their structural characteristics, the tubules and fibrils resemble the tubules in primary immunotactoid glomerulopathy and fibrils in primary fibrillary glomerulonephritis.
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PMID:Fingerprint and other organised deposits in lupus nephritis. 1102 Sep 62

Bowman's capsular and tubular basement membrane (TBM) deposits are an extremely unusual finding in non-lupus membranous glomerulopathy (MGN). We report three atypical cases of MGN with abundant Bowman's capsular and TBM deposits. In two cases, MGN was idiopathic; in the third case, MGN occurred in the renal allograft in the setting of HCV seropositivity. In addition to the usual glomerular capillary wall deposits, immunofluorescence and electron microscopy revealed extensive immune deposits within Bowman's capsule and TBMs, predominantly at the base of parietal and tubular epithelial cells. These cases suggest a potential pathomechanism of autoantibody to secreted epithelial antigens shared by visceral, parietal, and tubular epithelial cells. In all three cases, indirect immunofluorescence was unable to detect autoantibody to normal renal epithelial or matrix constituents. Furthermore, ELISA was unable to demonstrate circulating antibody to major extracellular matrix components. The implications of these findings for the pathogenesis of MGN are explored.
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PMID:Membranous glomerulopathy with Bowman's capsular and tubular basement membrane deposits. 1114 Aug 9

Lupus glomerulonephritis is a complication of systemic lupus erythematosus, with 10% of the patients developing end-stage renal disease. It is accepted that lupus patients are good candidates for kidney transplantation and that the disease activity is subdued after transplantation due to rigorous immunosuppression, with a low rate of graft loss due to recurrent glomerulonephritis. While recurrent fibrillary glomerulopathy has been reported in renal allografts, de novo disease has not. We report a patient with systemic lupus who underwent a renal transplantation and subsequently lost her allograft due to de novo fibrillary glomerulopathy. Four years after her first kidney transplant, the patient presented with acute deterioration of her renal function. A renal biopsy was performed, and it revealed a focal mesangioproliferative pattern with positive amorphous mesangial immunofluorescence staining for IgG and C3. Congo red staining was negative. Electron microscopy demonstrated the presence of randomly oriented nonamyloid fibrils in the mesangiun. The diagnosis of de novo fibrillary glomerulopathy was made. The patient lost her allograft and received a second cadaveric renal transplant 1 year later. She has had a stable renal function since then.
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PMID:De novo fibrillary glomerulopathy in the renal allograft of a patient with systemic lupus erythematosus. 1128 82


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