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At least 991 records · Page 55Linked to original sources
PubMed2001

Graft failure in a patient with systemic lupus erythematosus (SLE) treated with high-dose immunosuppression and autologous stem cell rescue.

A 32-year-old female with WHO grade IV, dialysis dependent, lupus nephritis was treated with high-dose immunosuppression and autologous stem cell rescue. Stem cells were mobilized with cyclophosphamide (CY) and G-CSF, and 4.07 x 10(6) CD34+ cells/kg were obtained after CD34+ cell selection using the CellPro column. The preparative regimen consisted of CY, and antithymocyte globulin (ATG), with methylprednisolone. After apparent primary engraftment of neutrophils on day 9, the patient developed recurrent neutropenia on day 19. She showed no evidence of engraftment by day 35, and back-up unmanipulated stem cells were given without effect. Subsequently, she received unmanipulated peripheral stem cells (2 x 10(6) CD34+ cells/kg) from an HLA-identical sibling. The patient remained pancytopenic and expired on day 62 from disseminated fungal infection. An autopsy revealed no evidence of hematopoietic recovery. Progenitor cell assays were performed with the patient's stem cells, which were collected prior to transplantation, and serum collected day 27. Morphologic examination of the patient's cell colonies grown in the presence of her serum revealed abnormal shapes and non-adherent cells. There were significantly fewer BFU-e colonies and a trend toward fewer CFU-GM colonies with the patient's cells and serum compared to normal donor cells. We concluded that a substance present in her serum mediated graft failure and prevented engraftment after additional stem cell infusions.

Adult↗
PubMed1999

Beneficial effect of the inosine monophosphate dehydrogenase inhibitor mycophenolate mofetil on survival and severity of glomerulonephritis in systemic lupus erythematosus (SLE)-prone MRLlpr/lpr mice.

The aim of the present study was to evaluate the therapeutic effect of mycophenolate mofetil (MMF) on the course of disease in SLE-prone MRLlpr/lpr mice. Three-months-old mice displaying clinical symptoms of glomerulonephritis were given MMF (100 mg/kg per day) orally via the drinking water. Control mice received i.p. injections of cyclophosphamide (CYC) (1.8 mg/mouse per week) or saline. Survival, albuminuria and haematuria, immunoglobulin levels and anti-dsDNA antibodies in serum, frequencies of immunoglobulin-producing B lymphocytes and glomerular deposits of immunoglobulin and C3 were analysed. The results showed that MMF treatment significantly prolonged survival and reduced the occurrence of albuminuria and haematuria in MRLlpr/lpr mice. In addition, the number of immunoglobulin-producing B cells and serum levels of IgG and IgG anti-dsDNA antibodies were reduced after MMF and CYC treatment. MMF treatment significantly reduced the extent of deposition of C3 in glomeruli. We conclude that the reduced severity of glomerulonephritis following treatment of lupus-prone mice with MMF was as efficacious as that of CYC. These results warrant clinical trials of MMF in SLE patients with glomerulonephritis.

Alkylating Agents↗
PubMedDate not supplied

Stevens-Johnson syndrome developing in a girl with systemic lupus erythematosus on high-dose corticosteroid therapy.

Stevens-Johnson syndrome (SJS) is a vesiculobullous disease of the skin and mucosa. This generalized hypersensitivity reaction is well known to occur in association with certain drugs, particularly sulfonamides, nonsteroidal anti-inflammatory agents (NSAIDs), and anticonvulsants. The disease is usually seen in children and young adults and is often treated with corticosteroids. We present a 9-year-old girl with lupus nephritis who developed cutaneous bullae and mucositis while being treated with intravenous methylprednisolone. The initial differential diagnosis included bullous lupus, but skin biopsy specimen findings supported a diagnosis of SJS. She was treated with intravenous immunoglobulin (IVIg).

Child↗
PubMed2005

Elevation of plasma osteopontin concentration is correlated with disease activity in patients with systemic lupus erythematosus.

BACKGROUND: Osteopontin (OPN) is an extracellular matrix cell adhesion phosphoprotein with immunological activities including stimulation of macrophage chemotaxis, T-helper type 1 lymphocyte response and B-cell antibody synthesis. Overexpression of OPN has been associated with the development of the autoimmune/lymphoproliferative syndrome. METHODS: We measured the plasma concentration and ex vivo production of OPN, and the plasma proinflammatory IL-18 concentration in 54 SLE patients with or without renal impairment (RSLE group and SLE group, respectively) and 26 sex- and age-matched control (NC) subjects using an enzyme-linked immunoabsorbent assay. RESULTS: Plasma OPN concentrations were significantly higher in RSLE and SLE patients than in the NC group (both P<0.001). Increase in OPN concentration correlated positively and significantly with SLEDAI score in all SLE patients (r = 0.308, P = 0.023). The ex vivo production of OPN upon mitogen activation of peripheral blood mononuclear cells was significantly higher in the RSLE and SLE groups than in the NC group (both P<0.001). In RSLE patients, plasma OPN concentration showed a significant positive correlation with proinflammatory cytokine IL-18 concentration (r = 0.404, P = 0.037). CONCLUSION: The above results suggest that the production of OPN is associated with the inflammatory process and SLE development, and may serve as a potential disease marker of SLE.

Adult↗
PubMed1993

Higher anti-heparan sulphate reactivity during systemic lupus erythematosus (SLE) disease exacerbations with renal manifestations; a long term prospective analysis.

Cross-reactive antibodies against heparan sulphate (HS) have been suggested to play a role in initiating renal disease in SLE. Recently, we found that HS-reactivity is mediated by anti-DNA antibodies complexed with DNA and histones. To evaluate the clinical significance of anti-HS reactivity, we studied prospectively a cohort of 72 consecutive SLE patients, of whom 22 experienced 40 exacerbations. In 20 of these exacerbations renal symptoms were present. In these 20 exacerbations significantly higher anti-DNA (median 1:160) and anti-HS (median 1:30) titres were detected compared with exacerbations without renal manifestations (median 1:60 for anti-DNA and negative for anti-HS). There were no correlations with other symptoms of SLE. Anti-HS titres showed a significant correlation with anti-DNA antibody titres (rs = 0.57, P < 0.05). Anti-HS without anti-DNA reactivity was never detected. Some SLE patients showed a high anti-DNA titre without anti-HS reactivity, suggesting that not all anti-DNA antibodies are able to bind to histone/DNA complexes and thus to exhibit anti-HS reactivity. Our findings indicate that anti-HS reactivity is correlated with renal disease in SLE.

Antibodies, Antinuclear↗