1/30. Haematopoietic stem cell transplantation for active systemic lupus erythematosus.OBJECTIVE: For patients with systemic lupus erythematosus (SLE) who are at risk of disease-related mortality, we have initiated a protocol of intensive immunosuppression and haematopoietic stem cell support. The first patient enrolled in this study was in the midst of a lupus flare manifest by nephritis and rapidly declining renal function, uncontrolled hypertension, immune-mediated cytopenias, and serositis characterized by a large pericardial effusion and abdominal pain. Antinuclear antibody (ANA), anti-double-stranded (ds) dna and complement were abnormal. This patient is now more than 1 yr post-stem cell transplant and is taking no immunosuppressive medication. Her serologies are normal, effusions have resolved, blood pressure is normal and renal function is markedly improved. The clinical and serological course of this patient is summarized here. methods: Autologous haematopoietic stem cells (HSC) were mobilized with cyclophosphamide (2.0 g/m2) and granulocyte colony-stimulating factor (G-CSF) (10 microg/kg/day). stem cells were enriched ex vivo using CD34-positive immunoselection and reinfused after immunosuppression with cyclophosphamide (200 mg/kg) and antithymocyte globulin (ATG) (90 mg/kg). RESULTS: White blood cell engraftment with an absolute neutrophil count (ANC) of >500/microl (0.5 x 10(9)/l) and platelet engraftment with a non-transfused platelet count of >20000/microl (20 x 10(9)/l) occurred on day 10 and 14, respectively. Therapy was complicated by a cell lysis-like effect with hyperphosphataemia, hyperuricaemia, normal anion gap metabolic acidosis and transient exacerbation of renal insufficiency. CONCLUSION: This is the first autologous T-cell-depleted haematopoietic stem cell transplantation performed to treat lupus in an active flare. This patient has, for the first time since discase onset (13 yr ago), entered a complete clinical and serological remission which persists at >1 yr of follow-up. The durability of this remission is unknown.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/30. Intravenous immunoglobulin therapy in a patient with lupus serositis and nephritis.The use of intravenous immunoglobulin (IVIg) has been reported as an immunomodulating agent in several autoimmune diseases, including systemic lupus erythematosus (SLE). Herein we report a SLE patient with severe clinical presentation that included pericarditis, pleural effusion, nephrotic range proteinuria, leukopenia, and lymphopenia. The patient received one course of high-dose IVIg (2.8 g/kg body weight), and within a week of post-IVIg therapy, her condition significantly improved. One-month post-IVIg there were decreased proteinuria, elevated leukocytes and lymphocytes count, decrease in antinuclear and anti-dsDNA antibodies, and disappearance of pericarditis and pleuritis. This case demonstrates the efficacy of IVIg in severe SLE with various clinical manifestations.- - - - - - - - - - ranking = 0.25051319059934keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/30. Antineutrophil cytoplasmic autoantibody specific for proteinase 3 in a patient with shunt nephritis induced by gemella morbillorum.A 17-year-old girl had been placed with ventriculoperitoneal, then ventriculoatrial shunts for congenital hydrocephalus since birth. The patient originally was diagnosed as having a lupus-like disease, but later turned out to have shunt nephritis, presenting with fever, proteinuria, pancytopenia, and hypocomplementemia. Antineutrophil cytoplasmic autoantibody specific for proteinase 3 (PR3-ANCA) was detected in her serum. The patient received oral prednisolone and repeated methylprednisolone pulses, with essentially no beneficial effects. A gram-positive coccus, gemella morbillorum, was recovered from her blood as well as cerebrospinal fluid, and the culture of the shunt catheter established the diagnosis of shunt nephritis. Removal of the shunt catheter improved symptoms dramatically and decreased PR3-ANCA in serum to an undetectable level. Because steroids had no effects and the control of bacterial infection lowered PR3-ANCA levels, the antibody would have been induced by continuous infection with G morbillorum.- - - - - - - - - - ranking = 0.01403144183315keywords = lupus (Clic here for more details about this article) |
4/30. De novo C1q nephropathy in the renal allograft of a kidney pancreas transplant recipient: bk virus-induced nephropathy?C1q nephropathy is a distinct entity characterized by extensive and dominant C1q mesangial deposition with associated steroid resistant proteinuria in the absence of systemic lupus erythematosus. Several morphological patterns ranging from very subtle glomerular alterations to focal/segmental glomerulosclerosis and mesangial proliferative changes have been described. Interstitial nephritis secondary to BK polyomavirus is a recently recognized complication in kidney transplant recipients. It may be associated with a tubulitis-like picture, mimicking sometimes acute tubular rejection. We report the case of a kidney pancreas transplant recipient who developed de novo C1q nephropathy, in the setting of BK polyomaviral interstitial nephritis. He presented with renal allograft dysfunction and a kidney biopsy was performed. It was interpreted as acute cellular rejection. C1q deposits were detected by immunofluorescence studies and electron microscopy. The patient did not respond clinically to appropriate anti-rejection treatment and a second renal biopsy was performed. The possibility of an interstitial nephritis secondary to BK polyomavirus mimicking rejection was suggested. Special immunohistochemical and blood/urine PCR studies for bk virus were performed, confirming the diagnosis of bk virus tubulonterstitial nephritis with a persistent, probable bk virus induced C1q nephropathy.- - - - - - - - - - ranking = 0.19438742326674keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
5/30. Antibody avidity for native dna in lupus nephritis.To study the participation of the antibody avidity in the pathogenesis of lupus nephritis, we measured the antibody avidity to native dna by the method of Minden and Farr (14) in patients with SLE. The avidity to native dna was almost less than 40%. The avidity and the histological activity of lupus nephritis were well correlated; the antibody avidity of the active-group sera was higher than that of the inactive-group sera and the group without nephritis. In the group with antibodies of relatively high avidity, the complexes were deposited mainly in the subendothelial side of GBM and in the mesangium. On the other hand, in the group with low-avidity antibodies, the complexes were localized in the subepithelial side of GBM. On investigating the quality of the complexes, we noted that the complexes composed of high-avidity antibodies prepared in vitro were larger than those of low avidity and the former were larger than 19S. If the native-dna-anti-native dna system is the mechanism basic to lupus nephritis, the differences in immune response of the host, namely the degree of antibody avidity may greatly affect complex formation and influence the histological activity and nephritogenicity of lupus nephritis.- - - - - - - - - - ranking = 0.1122515346652keywords = lupus (Clic here for more details about this article) |
6/30. Shunt nephritis: the nature of the serum cryoglobulins and their relation to the complement profile.The serum complement profiles of four patients with shunt nephritis indicated classical pathway activation of the complement system. The presence of mixed cryoglobulins was correlated with disease activity and the cryoglobulins were shown to be complement reactive. Antisera to two of the cryoglobulins recognized antigens of the infecting organism, and a specific bacterial antibody was identified in one cryoglobulin, giveing evidence that the cryoglobulins contained immune complexes. Bacterial antibody without detectable antigen was demonstrable in the sera indicating antibody excess. Renal morphology demonstrated mesangial proliferation and interposition with subendothelial and mesangial deposits. Parallels are drawn with active lupus nephritis.- - - - - - - - - - ranking = 0.01403144183315keywords = lupus (Clic here for more details about this article) |
7/30. Focal transient lesions of the corpus callosum in systemic lupus erythematosus.Focal lesions limited to the splenium of the corpus callosum are rare and little is known about their etiology. We describe three patients with systemic lupus erythematosus (SLE) that presented transient lesions of the corpus callosum. We reviewed three patients with SLE whose magnetic resonance imaging (MRI) results revealed focal lesions in the splenum of corpus callosum. The medical records, including clinical, serological, and treatment features, were reviewed to determine the etiology of these lesions. Of 115 patients who had MRI for research purposes, three patients with focal nonhemorrhagic lesions of the corpus callosum were identified. All patients had active SLE at the time of MRI. One patient had other findings on MRI, including cerebral venous thrombosis. On follow-up MRI, patients had an inactive disease and the corpus callosum lesions disappeared. A transient lesion in the splenium of corpus callosum seems to be a nonspecific endpoint of different disease processes leading to vasogenic edema. The complete and rapid reversibility in all cases with disease control is emphasized and any invasive diagnostic or therapeutic approach is discouraged.- - - - - - - - - - ranking = 0.9719371163337keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/30. levamisole in systemic lupus erythematosus.Two patients with systemic lupus erythematosus complicated with lupus nephritis were treated with levamisole, an immunomodulator. Clinical features, including urinary protein excretion and creatine clearance, were restored in one patient, also immunoparameters such as ANA, Ig-bearing cell number and PHA-skin test. The other patient did not respond to levamisole but did respond to cyclophosphamide.- - - - - - - - - - ranking = 0.98596855816685keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/30. Systemic lupus erythematosus with nephritis in children. A report of 6 cases.Six children with systemic lupus erythematosus treated over the last 10 years are described. There are 3 boys and 3 girls and their ages at presentation ranged from 9.25 to 15 years; follow-up ranges from 11 months to 12 years. The modalities of treatment, methods of follow-up, and regular assessment of lupus activity are discussed with a brief review of the relevant literature. One patient has progressed to end-stage renal failure and is awaiting renal transplantation. Of the others, 2 have mild renal impairment and 3 have normal renal function. We believe that an aggressive therapeutic approach with meticulous follow-up can improve the outlook in children with this condition.- - - - - - - - - - ranking = 0.84216655033004keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/30. Rapid improvement in severe lupus glomerular lesions following intensive plasma exchange associated with immunosuppression.We report our experience of intensive plasma exchange (PE) in the treatment of 12 females with severe diffuse proliferative lupus nephritis. All had active disease with crescentic lesions demonstrated on biopsy immediately before PE. Nine patients were also treated with high dose steroids, three patients with low dose steroids and most patients also received cytotoxic therapy. Eight of the 12 patients were biopsied immediately after a course of PE. All but one patient (low dose steroid group) showed considerable diminution of histologic activity with resolution of crescentic lesions. plasma exchange may accelerate such resolution over conventional therapy, prevent subsequent sclerosis and preserve functional renal tissue.- - - - - - - - - - ranking = 0.070157209165751keywords = lupus (Clic here for more details about this article) |
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