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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An idiopathic nephrotic syndrome associated with membranous glomerulopathy antedated the subsequent emergence of systemic lupus erythematosus in two patients (7-year-old and 14-year-old girls). At the onset of INS, there was neither clinical evidence of multisystem disease nor unequivocal serologic evidence of SLE. The only early possible indication of SLE was the presence of microtubular inclusions in glomerular endothelial cells on electron microscopy. In each instance (one year and three years after onset of INS), a second renal biopsy showed transformation of the membranous glomerular lesion to a more florid type with glomerular subendothelial dense deposits. One patient died of overwhelming pulmonary infection while she was receiving prednisone and cyclophosphamide; the other developed progressive renal failure despite steroid treatment. SLE should be considered in patients presenting with apparent idiopathic MG, in whom nephrotic syndrome persists. Intraendothelial cell microtubular inclusions may be an early clue to later emergence of SLE.
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PMID:Idiopathic membranous glomerulopathy preceding the emergence of systemic lupus erythematosus in two children. 83 Aug 97

Lupus anticoagulant (LAC) is an acquired inhibitor of prothrombin activator complex, which probably interferes with the phospholipid portion. Characteristically, LAC prolongs the partial prothrombin time, but only slightly prolongs the prothrombin time. It is a paradoxical fact that LAC is characterized by thrombosis. It was initially described in patients with SLE, but recently, it has been described as occurring with other autoimmune disorders. Patients with LAC have been treated with steroid and aspirin, anti platelet agents or warfarin. Steroid and aspirin therapy has been reported useful for habitual abortion associated with LAC. In our study, 11 patients, whose prior pregnancies resulted in habitual abortion (41 abortions), received intentional prednisolone (40 mg/day) and aspirin (81 mg/day) therapy before further pregnancies. The doses of both agents were decreased gradually, and the therapy with prednisolone (10-15 mg/day) and aspirin (40.5 mg/day) was maintained during the pregnancy period. The outcome of pregnancy was successful in 7 out of 10 pregnancies. To evaluate the relationship between LAC and glomerulopathy, we examined the renal biopsy from 5 LAC cases without SLE. In pathological findings, there were 3 of with mild proliferative GN and 2 cases of minor glomerular abnormality. There were no characteristic findings in LAC nephropathy.
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PMID:[Lupus anticoagulant]. 130 2

Renal transplant biopsies were obtained from 16 patients with systemic lupus erythematosus 6 months to 11 years post-transplant. Eight biopsies were taken on clinical grounds while eight were elective. Histopathological findings suggesting recurrent lupus nephritis were found in seven biopsies, five of which were taken on clinical indication. By light-microscopy, five graft biopsies showed proliferative glomerulopathy and two glomerulosclerosis. Immunofluorescence was positive for IgM and C3 in a finely granular pattern in all biopsies, for C1q in three, but for IgG in only two. Electron-dense deposits were found in all seven biopsies with predominantly subendothelial location. All but one patient had clinical signs of renal involvement, but only three had extrarenal symptoms and three had serological signs of active SLE. Upon increased immunosuppressive therapy, renal and serological signs improved but one graft was later lost due to recurrent SLE nephritis.
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PMID:Recurrence of SLE in transplanted kidneys: a follow-up transplant biopsy study. 133 39

A previously healthy 34-year-old woman, was diagnosed as having systemic lupus erythematosus (SLE), with membranous glomerulopathy which improved rapidly. Neither lupus anticoagulant nor anticardiolipin antibodies were detected in her plasma. After three months of total remission, she developed a severe pulmonary thromboembolism for which no specific biological cause was found. Her plasma was analysed for different antiphospholipid antibodies: lupus anticoagulant and anticardiolipin antibodies were again negative. Using an ELISA prepared with either five different anionic phospholipids or zwitterionic phosphatidylethanolamine, solely an anti-phosphatidylethanolamine IgG was discovered in her plasma. In lupus patients, the presence of antiphospholipid antibodies is now well recognized as a high risk factor for repeated thrombosis and/or recurrent abortions. This case suggests that the presence of antiphosphatidylethanolamine antibody should be investigated in cases of unexplained thrombosis in SLE, where the usual clinical and biological investigations have failed to shed light.
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PMID:Antiphosphatidylethanolamine antibody as the sole antiphospholipid antibody in systemic lupus erythematosus with thrombosis. 148 13

A male aged 58 was admitted to our hospital because of proteinuria, hematuria and bilateral pretibial edema. Laboratory tests showed normocytic, normochromic anemia and moderately impaired renal function. Antinuclear antibodies were negative. Neither M-protein nor Bence-Jones protein were detected. Light microscopic study on the biopsied renal specimen indicated a moderate mesangial proliferation accompanying with the deposition of PAS-positive and Congo red-negative materials in the subendothelial area. C3 accumulated segmentally along the capillary walls, which was clarified by immunofluorescence microscopy. Staining for IgG, IgA, IgM and light chains were negative. Electron microscopy demonstrated the deposition of microtubules in the mesangial, subepithelial and subendothelial areas. The diameter of these microtubules ranged from 40 to 80 nm. Such type of the microtubules have been reported to exist in the glomeruli in the patients with systemic diseases such as amyloidosis, systemic lupus erythematosus, cryoglobulinemia and light chain disease. In our patient, however, any clinical or serological findings suggestive of these systemic diseases were not obtained. On the other hand previous report pointed out that microtubules deposited in the glomeruli in the patients with immunotactoid glomerulopathy or other glomerulopathies. Our patient had the clinical features consistent with these glomerulopathies. However, no depositions of immunoglobulins were observed. This case is an atypical glomerulopathy accompanying with the glomerular microtubular deposits.
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PMID:[Glomerulopathy associated with glomerular microtubular deposits: a report of a case]. 148 13

Pediatric cases of systemic lupus erythematosus with an unfavorable outcome (terminal renal failure requiring chronic hemodialysis, or death) assembled during a retrospective multicenter study of pediatric SLE in the Paris metropolitan area were analyzed. Seven patients (6 girls, 1 boy) were entered into a chronic hemodialysis program. Four had diffuse proliferative glomerulonephritis, the pattern of glomerular disease classically responsible for end-stage renal failure. The other three patients had membranous glomerulonephritis with active segmental lesions, a form of glomerulopathy whose severe prognosis deserves to be emphasized. Nine other patients (8 girls, 1 boy) died. In six patients, death occurred as a result of a flare with malignant hypertension and progressive renal failure (1 case), pancreatitis (1 case), encephalopathy (2 cases) or cardiomyopathy (2 cases). An infectious disease (tuberculosis, mumps) was apparently the cause of the two cases of encephalopathy. One girl died as a result of a hemorrhagic syndrome with a cerebral hematoma. Two other girls died at home. Overall, among 111 children with SLE 14% had an unfavorable outcome. Sex and age at onset seemed to have no bearing on prognosis. Patients with renal involvement were apparently more likely to have an unfavorable outcome. Lastly, although the influence of ethnic origin is unproven, children living in foreign countries of French overseas territories, but treated in France have an increased risk for unfavorable outcomes.
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PMID:[Unfavorable outcomes in disseminated lupus erythematosus in children. Cooperative study in the Paris region]. 192 11

During the past 10 years, immunotactoid glomerulopathy has become recognized with increasing frequency. The lesion is characterized histologically by highly organized ultrastructural deposits that appear to be composed of immunoglobulin and complement and are negative for amyloid by Congo red stain. Clinically and/or serologically, patients have no evidence of cryoglobulinemia, amyloidosis, systemic lupus erythematosus, or a paraproteinemia, disorders associated with glomerular deposits, which also have a highly organized tactoidal or fibrillar characteristic. Immunotactoid glomerulopathy does not appear to be a multisystemic disease process and thus may represent a primary glomerulopathy. Patients with immunotactoid glomerulopathy present with proteinuria (nephrotic range in more than 60%) and over half of the patients have hypertension, hematuria, and renal insufficiency. Progression to end stage renal disease has occurred in more than 40% of patients reported to date. The experience in treating this disorder using prednisone and/or immunosuppression is limited and has not been impressive. Four patients have successfully undergone renal transplantation, but proteinuria recurred in two and was associated with the recurrence of immunotactoid glomerulopathy in the renal allograft. Although we have gained insight into the clinical course and histopathology of this disorder over the past few years, we still know little about its pathogenesis, an area for further research.
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PMID:Immunotactoid glomerulopathy. 199 64

Excretion patterns of kidney related urinary proteins such as lysosomal beta-N-acetylglucosaminidase (beta NAG), brush-border Ala-(Leu-Gly)-aminopeptidase (AAP), gamma-glutamyl transpeptidase (GGT), and alkaline phosphatase (AP) as well as of IgG, albumin, and alpha-1-microglobulin, were assessed in patients with chronic glomerulonephritis (n = 53), pyelonephritis (n = 27), systemic lupus erythematodes (n = 5), and patients with essential arterial hypertension (n = 18). Excretion of tubular marker enzymes and serumproteins (related to urine creatinine concentration = protein creatinine index) in spontaneously voided second morning urine was significantly higher as compared to the controls (n = 2). Alpha-1-microglobulin was markedly elevated in both pyelonephritis and glomerulonephritis indicating disturbance in tubulointerstitial handling of microglobulins also in cases with primary glomerulopathy. Rise of albumin, IgG, and alpha-1-microglobulin as well as of tubular kidney markers AAP, AP, GGT, and beta NAG in cases with arterial hypertension without preexisting nephropathy support the hypothesis of a defect in charge and size permselectivity in these patients which is probably due to an increase in glomerular capillary perfusion pressure and hyperfiltration.
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PMID:Kidney- and serum derived proteins in urine of patients suffering from renal diseases or arterial hypertension. 247 9

Immunotactoid glomerulopathy is a distinct clinico-pathological entity which has recently been defined. The term immunotactoid refers to highly organized immune depositions appearing as rod-like microtubular structures in ultrastructural examination. We describe a patient with mixed connective tissue disease who demonstrates characteristic features of immunotactoid glomerulopathy. The diagnosis was made after excluding amyloidosis, cryoglobulinaemia and lupus nephritis. In addition to immunotactoid microtubules, ultrastructural examination also demonstrated presence of fingerprint depositions which were intimately mixed with immunotactoid structures. Fingerprint deposits have been described in lupus nephritis and cryoglobulin-related nephropathy, but rarely in other glomerulonephritis. These unique findings in our patient may suggest a previously unsuspected relationship between the syndrome of immunotactoid glomerulopathy and systemic lupus erythematosus.
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PMID:Immunotactoid glomerulopathy with fingerprint immune deposits. A variant of lupus nephritis? 250 Jul 73

A total of 411 children, aged from 0.3 to 18 years, suffering from glomerular diseases, were studied by renal biopsy between 1976 and 1985. The clinical presentation included nephrotic syndrome (79% of cases), renal failure (43%), and arterial hypertension (38%). In all, 177 cases presented with primary nephrotic syndrome; all had complicated courses and most were either corticosteroid-dependent or -resistant. Only 26.6% had minimal change disease on renal biopsy; 56.5% had focal-segmental sclerosis; and immunofluorescent deposits were observed in half of the group. Acute poststreptococcal (36 cases), mesangiocapillary (80 cases), and lupus (34 cases) glomerulonephritis occurred frequently; IgA glomerulopathy (10 cases) and haemolytic uraemic syndrome (6 cases) were uncommon. Glomerular crescents were observed in 71 cases. These observations illustrate the types of glomerular diseases seen in Iranian children.
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PMID:Glomerular diseases in children. "The Iranian experience". 270 71


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