1/139. Glomerular thrombosis: an unusual cause of renal failure in systemic lupus erythematosus.The authors report an unusual case of acute renal failure occurring in a patient with systemic lupus erythematosus and antiphospholipid antibodies. kidney biopsy revealed glomerular thrombosis, in the absence of glomerulonephritis. The authors stress the clinical and biological signs that suggest the thrombotic nature of kidney failure in lupus patients.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/139. Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus.Two patients with systemic lupus erythematosus are described who in the course of their disease developed small atrophie blanche lesions that closely resembled those found in malignant atrophic papulosis. Preliminary investigation of these two cases indicates that considerable similarities probably exist in the pathogenesis of malignant atrophic papulosis and the atropie blanche lesions of systemic lupus erythematosus. It is concluded that a diagnosis of malignant atrophic papulosis should only be made after systemic lupus erythematosus has been excluded by full investigation.- - - - - - - - - - ranking = 1.374923250759keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/139. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 0.28597742596928keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/139. Bilateral renal vein and inferior vena cava thrombosis with nephrotic syndrome treated by thrombectomy: case report with 3-year followup.A previously healthy 19-year-old woman taking anovulatory medication presented with symptoms of the nephrotic syndrome and lupus erythematosus. diagnosis of inferior vena cava and bilateral renal vein thrombosis was made angiographically. The patient was treated successfully by thrombectomy and anticoagulation, and remains well 3 years later. Laboratory data indicate normal renal function and only mild proteinuria. This is the longest followup of a patient with this entity reported in the literature.- - - - - - - - - - ranking = 0.16075867570356keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
5/139. A case of antiphospholipid antibody syndrome diagnosed after thrombosis of an arteriovenous shunt.A 32-year-old male dialysis patient with lupus nephritis was admitted because of shunt obstruction. The arteriovenous fistula was reconstructed, but obstruction recurred twice within several hours after surgery. A high blood level of anticardiolipin beta2-glycoprotein I antibody suggested that shunt obstruction was caused by a thrombotic tendency related to the antiphospholipid antibody syndrome. Accordingly, for the third shunt procedure, antiplatelet therapy (which had been commenced for systemic lupus erythematosus) was combined with dalteparin sodium from before surgery and warfarin was added postoperatively. This regimen prevented shunt obstruction. In conclusion, hemodialysis patients who suffer repeated shunt obstruction should be examined for antiphospholipid antibody syndrome.- - - - - - - - - - ranking = 0.21432957099488keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
6/139. A case of primary antiphospholipid antibody syndrome with acute renal failure showing thrombotic microangiopathy.An 18-year-old woman complained of fever and edema and was admitted to Showa University Hospital for treatment of thrombocytopenia and deteriorating renal function. Laboratory studies demonstrated the presence of lupus anticoagulant (LA), prolongation of prothrombin time, hemolytic anemia, a negative Coombs' test, the absence of antinuclear antibodies, and a normal fibrinogen level. Renal biopsy revealed mesangial hypercellularity, severe endocapillary cell damage, and double contour of the basement membrane walls. Immunofluorescence studies demonstrated focal, peripheral, and finely granular deposits for IgG, IgM, and IgA but were negative for fibrinogen. Electron microscopy showed glomerular capillary loops with subendothelial widening and subendothelial deposits, mesangiolysis, mesangial interposition, and marked luminal narrowing. biopsy findings were consistent with thrombotic microangiopathy. The patient was treated with hemodialysis, methylprednisolone pulse therapy, and dipyridamole. After treatment, LA disappeared, the prothrombin time became normal, and renal function improved. The renal lesions in this patient were caused by primary antiphospholipid antibody syndrome. This case strongly suggests an important causal relationship between LA and renal lesions in thrombotic microangiopathy. We present this case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 0.017911963743599keywords = lupus (Clic here for more details about this article) |
7/139. Reactive angioendotheliomatosis in association with the antiphospholipid syndrome.A 50-year-old woman with systemic lupus erythematosus developed extensive necrotic skin lesions on her chest and abdomen after the discontinuation of warfarin. The presence of antiphospholipid antibodies suggested a diagnosis of antiphospholipid syndrome. Histopathology from a skin lesion demonstrated marked expansion of the dermal microvasculature by intravascular cellular proliferation and focal thrombosis. The intravascular cells stained positive for the endothelial cell markers CD31 and factor viii-associated antigen confirming reactive angioendotheliomatosis (RAE). This report is the first to identify RAE occurring in the context of the antiphospholipid syndrome. We suggest that intravascular endothelial proliferation, in concert with thrombosis, contributed to the angio-occlusive pathology.- - - - - - - - - - ranking = 0.19641760725128keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/139. Acute brachial artery thrombosis as the initial manifestation of human immunodeficiency virus infection.Thrombosis of upper extremity arteries is most commonly due to atherosclerosis of the proximal subclavian artery, trauma, or catheter-related injury. In the absence of an identifiable cause, a search for a hypercoagulable state is indicated. Hematologic manifestations of human immunodeficiency virus (hiv) infection and AIDS are frequent occurrences (Coyle TE. Med Clin N Am 1997;81:449-476). The most important of these are cytopenias (anemia, neutropenia, and thrombocytopenia). The incidence and severity of cytopenia are generally correlated to the stage of the hiv infection. In addition, various coagulation abnormalities have been reported in hiv-infected patients. Apart from thrombocytopenia, these have included a prolonged APTT due to the presence of lupus anticoagulant, an increased prevalence of protein S and heparin cofactor ii deficiency, and hypoalbuminemia-related fibrin polymerization defects (Toulon P. Ann Bio Clin (paris) 1998;56:153-160). hiv infection has also been associated with endothelial dysfunction. Although for the most part asymptomatic, elevated D-dimer levels have been found in hiv-infected patients, suggesting the existence of a prethrombotic state. In fact, clinical thrombosis eventuates in 2% of these patients (Toulon, 1988). Documented thromboses have involved both veins and arteries. We hereby present a patient who developed an acute thrombosis of his brachial artery as the initial manifestation of hiv infection.- - - - - - - - - - ranking = 0.017911963743599keywords = lupus (Clic here for more details about this article) |
9/139. "Pauci-Immune" proliferative and necrotizing glomerulonephritis with thrombotic microangiopathy in patients with systemic lupus erythematosus and lupus-like syndrome.In the glomerulonephritides of systemic lupus erythematosus (SLE), the number of subendothelial deposits, when present, generally corresponds to the degree of light microscopic glomerular hypercellularity; only very rarely are no or few such deposits present in cases of focal (WHO class III) or diffuse (WHO class IV) proliferative lupus nephritis. We have recently encountered five cases of active diffuse proliferative glomerlonephritis with no subendothelial and few or no mesangial deposits and thrombotic microangiopathy (TMA) in four patients with SLE and one patient with lupus-like syndrome. Three of the five patients were tested for circulating lupus anticoagulants or anticardiolipin antibodies, and two were positive. All five patients tested negatively for antineutrophil cytoplasmic antibodies (ANCA). Three patients responded to steroid and cyclophosphamide treatment, although one of them died of acute bacterial bronchopneumonia. One patient was lost to follow-up. We conclude that "pauci-immune" proliferative lupus nephritis is rare and should be treated as proliferative lupus nephritis with a proportionate number of subendothelial deposits. The negative ANCA suggests that these cases do not represent incidental ANCA-associated pauci-immune necrotizing and crescentic glomerulonephritis in patients with SLE. Of particular interest is that, in patients with SLE, if associated with TMA, an active proliferative necrotizing glomerulonephritis may be present even in the absence of significant glomerular immune complex deposition.- - - - - - - - - - ranking = 1.1432957099488keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/139. Peripheral arterial thrombosis in systemic lupus erythematosis and nephrotic syndrome: possible association with protein s deficiency.Arterial thrombosis in systemic lupus erythematosus (SLE) and nephrotic syndrome have been infrequently reported. A 16-year-old boy with SLE and longstanding nephrotic syndrome presented with peripheral arterial thrombosis when his lupus was at an inactive stage. He did not have antiphospholipid antibodies but had low serum antithrombin iii and protein S levels. We suggest that the thrombotic event is not related to antiphospholipid antibodies but to nephrotic syndrome and possibly to acquired protein s deficiency.- - - - - - - - - - ranking = 0.31482471561152keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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