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)

To investigate the role of the complex IgA-alpha-1-antitrypsin (IgA-AT) in systemic lupus erythematosus (SLE) and in mixed connective tissue disease (MCTD), and its possible relations to either activity of the disease or a treatment, we examined a concentration of IgA-AT complex in 65 SLE and 9 MCTD sera. Complex IgA-AT was evaluated using a double antibody enzyme-linked immunoassay (ELISA). Twenty nine patients with SLE (44.6%) and three patients with MCTD (33.3%) had increased serum IgA-AT levels. The mean values of IgA-AT complex in patients with SLE and MCTD were higher than in healthy controls. Among the SLE group, patients with current neurological manifestation were characterized by an increase in IgA-AT serum concentration (2.45 +/- 2.07 U vs. 0.78 +/- 0.70 U, P < 0.001). No relation was found between this complex and ESR level, C-reactive protein (CRP) concentration, or hemoglobin level. Ten SLE patients were treated with CTX intravenously. In this group of patients, IgA-AT complex level was found to be increased compared with patients without such a treatment (1.82 +/- 1.30 U vs. 0.80 +/- 0.67 U, P < 0.05). The present study provides two new observations. Firstly, IgA-AT complex is increased in SLE and MCTD compared with healthy controls, and secondly, patients with CNS involvement displayed a striking increased IgA-AT level.
Lupus 1995 Jun
PMID:IgA-alpha-1-antitrypsin complex in systemic lupus erythematosus: preliminary report. 765 94

A female patient with a history of migraines and chorea developed polyarthralgia at age 24 and was diagnosed with rheumatoid arthritis. In 1991 she was hospitalized because of impaired renal function and hypertension. Examination revealed thrombocytopenia and the presence of lupus anticoagulant. Antinuclear antibody was weakly positive, but anti-DNA antibody was negative, and no decrease in leukocyte count or complement level was observed. Rheumatoid arthritis with antiphospholipid syndrome was diagnosed. Renal biopsy showed renal thrombotic microangiopathy. This renal lesion was considered to be associated with antiphospholipid syndrome. Cyclophosphamide pulse therapy and anticoagulation therapy decreased proteinuria and improved renal function.
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PMID:Renal thrombotic microangiopathy in a patient with rheumatoid arthritis and antiphospholipid syndrome: successful treatment with cyclophosphamide pulse therapy and anticoagulant. 780 16

A patient with systemic lupus erythematosus developed unexplained fever, nonregenerative anemia, leukopenia, and elevations in serum triglyceride and ferritin levels. Bone marrow studies established the diagnosis of macrophage activation syndrome with active hemophagocytosis. No infectious cause was found but pulmonary nocardiosis developed during the course of the disease. Intravenous gammaglobulin therapy was followed by a transient remission. Cyclophosphamide was given subsequently. In lupus patients, macrophage activation syndrome is exceedingly rare and has the same clinical, laboratory, and histologic features as those seen in patients with hemopathies, infections, or immune deficiencies. Investigations for an underlying infection are often negative, suggesting that the macrophage activation syndrome is due to lupus-related immune changes. Treatment is not standardized and relapses are common. This diagnosis should be considered in lupus patients with febrile pancytopenia.
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PMID:[Macrophage activation syndrome in lupus]. 805 32

Pulmonary hypertension was diagnosed in a patient with SLE after her 5th delivery. Time of onset and absence of thromboembolism and severe interstitial lung disease suggested primary pulmonary hypertension. Administration of vasodilators did not decrease pulmonary artery pressure which amounted to 82/30 mm Hg. Cyclophosphamide infusions, 0.5 g/m2 monthly for 6 months followed by once/3 months, in combination with 7.5 mg prednisolone daily resulted in decrease of pulmonary artery pressure to 66/34 mm Hg after 6 months of treatment persisting for 30 months. Intermittent infusions of cyclophosphamide with low dose prednisolone may be effective in primary pulmonary hypertension in SLE, suggesting an immune mediated pathogenesis.
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PMID:Primary pulmonary hypertension in a patient with systemic lupus erythematosus: partial improvement with cyclophosphamide. 783 74

We report the clinical case of an 8 years female with systemic lupus erythematosous who developed transverse myelitis secondary to antiphospholipid syndrome. She had an excellent response to the treatment with Prednisone and Cyclophosphamide. As long as we know this is the first report of transverse myelitis as clinical manifestation of antiphospholipid syndrome in childhood.
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PMID:[Transversal myelitis as initial manifestation of secondary antiphospholipid syndrome. Report of a case]. 852 83

To evaluate the clinical effectiveness of intermittent intravenous cyclophosphamide in the treatment of severe systemic lupus erythematosus, 20 patients with systemic lupus erythematosus (SLE) and evidence of severe renal involvement or systemic vasculitis, consecutively admitted to the hospital were studied. Cyclophosphamide was administered intravenously at a dosage of 1.0 g/m2 monthly, during 6 months and maintained every 3 months during 12 additional months. Of 10 patients with active lupus nephritis, a reduction or disappearance of proteinuria and maintenance of normal renal function was recorded in 6. Improvement of renal function was observed in 4 out of 7 patients with renal insufficiency at initial evaluation; resolution of renal insufficiency was more frequently observed in patients with recent onset renal failure. At the end of the follow-up (18.0 +/- 14.5 months) disappearance or reduction of nephrotic range proteinuria was recorded in 6 out of 14 patients; there was progression toward renal failure in 4 patients (20%). Response to intravenous cyclophosphamide therapy was observed in 4 of 5 patients with severe extrarenal SLE. Side effects, recorded in 12 patients, were mild and transient and in no patient was the treatment discontinued. Four patients died during the follow-up, although in 2 of them the deaths were not attributable to therapy. Even though this was an open and uncontrolled study, intermittent, intravenous cyclophosphamide was an effective therapy for severe, steroid refractory SLE.
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PMID:Clinical effects of intermittent, intravenous cyclophosphamide in severe systemic lupus erythematosus. 889 47

Immunosuppressor and immunomodulator therapies are widely used for the treatment of patients with severe systemic lupus erythematosus. In case of certain organ involvement (kidney, brain, heart), corticosteroids should be associated with immunosuppressors, especially cyclophosphamide. Cyclophosphamide, a powerful immunosuppressor, is generally given in an intravenous bolus rather than orally. Aziathioprine is proposed after cyclophosphamide or in less forms at onset. The therapeutic strategy in severe forms may require plasma exchange, carefully synchronized with the cyclophosphamide bolus. Depleted antibodies following plasma exchange induces a stimulation of B clones which produce antibodies sensitive to cyclophosphamide. Other immunomodulator treatments have also been used, for example cyclosporine or intravenous immunoglobulins. Cyclosporine has not been shown to be effective in severe lupus. Inversely, in certain conditions, cyclosporine can be used to limit other treatments although it does not have a significant effect on autoantibody levels. Intravenous immunoglobulins have been used at high dosages but only in selected cases due to undesirable side effects. Certain cases of renal failure induced by immunoglobulins have been described. Immunosuppressor or immunomodulator therapy is often required in systemic lupus erythematosus. Treatment modalities must however be carefully established in order to limit the predictable side effects while achieving maximal efficacy.
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PMID:[Immunosuppressive and immunomodulator treatment of severe systemic lupus]. 909 61

Cyclophosphamide is a potent immunosuppressive drug which is widely used to treat renal and central nervous system manifestation of Systemic Lupus Erythematosus (SLE). It is employed especially to prevent renal failure in lupus nephritis. Within the last decade intravenous cyclophosphamide bolus therapy has become the standard therapy in severe SLE due to its tendency towards a higher efficacy and fewer side effects as compared to oral application. Studies on the slightly more cost-effective oral cyclophosphamide bolus therapy have recently been published, however, there are no data on long-term results yet. Here, we review the effects and side effects of cyclophosphamide as well as recent clinical studies on cyclophosphamide bolus therapy in SLE. Because of the possible side effects, especially the high risk of malignancies, which sharply increases at a cumulative dose of approximately 60 g, cyclophosphamide should be used cautiously. Cyclophosphamide bolus therapy should only be performed in hospitals with special experience.
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PMID:[Cyclophosphamide therapy in systemic lupus erythematosus]. 941 58

Cytotoxic therapy, especially with cyclophosphamide in the dose 8-20 mg/kg used as intermittent pulses, has been shown to improve both patient and renal survival in systematic lupus erythematosus (SLE), but to date there is no cure for the disease. Owing to the paucity of recognisable clones, the rationale and goal of cytotoxic immunosuppressive therapy in the treatment of immune-mediated diseases as against malignancies is to suppress the aberrant inflammation and immune-mediated reactions responsible for tissue damage, without dangerously suppressing the normal host defence mechanism(s). We report the case of a patient suffering from SLE with nephritis who has remained in sustained remission over the past 8 years without any maintenance therapy following an accidental administration of a single dose of 5000 mg of intravenous cyclophosphamide (44.2 mg/kg body weight). The patient recovered fully from pancytopenia following the injection. Presently, she is asymptomatic and working gainfully. Her laboratory parameters including blood counts, urine analysis, FANA and anti-dsDNA have reverted to normal. Cyclophosphamide in the dose of 30-160 mg/kg has been safely and effectively used in various neoplastic conditions with the aim of destroying every possible tumour cell. The experience of the present case suggests that such an approach may be applicable to SLE.
Lupus 1999
PMID:'Sustained remission' in a case of SLE following megadose cyclophosphamide. 1048 38

A 35 y old woman with severe and progressive systemic lupus erythematosus (SLE) received high-dose chemotherapy followed by a T cell depleted autologous stem cell transplantation. Peripheral blood stem cell were mobilised with Cyclophosphamide 4.5 g/m2 followed by Granulocyte-Colony Stimulating Factor (G-CSF). A CD34 positive selection provided a 3 log T cell depletion. High-dose immunosuppression consisted of the BEAM regimen. The purified autograft was reinfused on day 0. In the post transplant period, hemopoietic growth factors, G-CSF, Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) and Erythropoietin, were administered, engraftment was rapid. Both the mobilisation and the transplant procedures were easily performed and well tolerated. One year later, the patient is in clinical remission. The ANA and anti-SSA-antibodies were undetectable at 1 and 6 months after intensification, but reappeared at low levels at 9 months. Corticosteroid requirement has gradually decreased. In conclusion, we report here the favourable evolution of a patient with a severe SLE, who clinically improved with high-dose immunosuppressive therapy and autologous stem cell transplantation, and showed a 9 month serological remission.
Lupus 1999
PMID:Control of severe systemic lupus erythematosus after high-dose immunusuppressive therapy and transplantation of CD34+ purified autologous stem cells from peripheral blood. 1041 12


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