Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

At its inception, morphological imaging, like magnetic resonance imaging (MRI), is often not useful in neuropsychiatric systemic lupus erythematosus (NPSLE), although the disease clinically shows cerebral symptoms. Functional imaging, like positron emission tomography (PET), may be a method that offers some advantages. We report a 53-year-old white man with decreased memory and visual disturbances who met four of the American Rheumatism Association (ARA) criteria for the classification of SLE. He was investigated before and after 3 months of therapy using PET and F-18-fluoro-2-deoxy-D-glucose (FDG). Treatment consisted of prednisone (25 mg/day, tapered to 10 mg/day) and cyclophosphamide (daily 100 mg for 3 weeks followed by a drug-free interval of 1 week). For the control group, 15 clinically and neurologically healthy volunteers (5 male, 10 female, aged 48 +/- 7 years) were investigated. All study participants additionally had a cranial MRI. In both controls and the SLE patient, cranial MRI was negative. However, the patient showed a significant hypometabolism in the region parieto-occipital on both sides and the parietal region on the right side before treatment. After treatment metabolism in these regions was within normal limits. Hence, FDG-PET could help to verify brain-onset of SLE earlier and may be a powerful tool for controlling SLE treatment.
Lupus 1998
PMID:Neuropsychiatric systemic lupus erythematosus before and after immunosuppressive treatment: a FDG PET study. 949 52

Immunoablation followed by allogeneic stem cell (SC) transplantation has been shown to be capable of curing a large spectrum of experimental autoimmune disorders, hereditary and/or induced. Superimposable results, albeit with some exceptions, have been obtained in human patients affected by coincidental autoimmune and blood diseases. However, both because of encouragine experimental results and of the procedure's greater safety, autologous SC are being increasingly utilized worldwide. Case reports are being collected in the registry of the European Group for Blood and Marrow Transplantation (EBMT)/European League against Rheumatism (EULAR) Autoimmune Disease Stem Cell Project. Among the severe autoimmune diseases (SADs), which are the target of autologous transplantation, severe refractory systemic lupus erythematosus (SLE) is a condition which may benefit from this procedure. We report here the case of a 19 year old female patient with a six year history of SLE with secondary antiphospholipid syndrome (APS), who later developed refractory Evans syndrome. She was transplanted with autologous mobilized CD34+ SC and progenitor cells after conditioning with cyclosphosphamide, anti-T lymphocyte globulin and prednisone. Eight months after transplant, the patient is alive and well, with normal blood counts and persistent low-titre direct antiglobulin (DAT, Coombs) and anti-nuclear antibody (ANA) tests. Anti-double stranded DNA antibody (Anti-dsDNA), lupus anticoagulant tests and anti-cardiolipin antibody (ACA) test are negative.
Lupus 1998
PMID:Autologous peripheral blood stem and progenitor (CD34+) cell transplantation for systemic lupus erythematosus complicated by Evans syndrome. 979 53

The 40-year experience of the Institute of Rheumatology has shown that follow-up in rheumatic diseases is the necessary method of management of patients with rheumatic diseases since it is the method that makes it possible to control the progression of disease and provide long-term safe treatment with glucocorticoids, cytotoxics, and other antirheumatic drugs which are now widely used in rheumatological care. Medical examination mainly made in rheumatism caused a substantial reduction in the number of relapses. Since the 1960s, intensive and scientifically grounded studies in medical examinations for other rheumatic diseases have promoted more prolonged longevity and better working fitness in the vast majority of patients. Thus, a long-term follow-up of over 600 patients with exanthematous lupus erythematosus made it possible to detail its clinical picture and to develop the therapeutical programmes that control the progression of the disease in 93% of the patients who had been incurable before. In rheumatoid arthritis, the follow-up of large groups of patients by using the computer database has enabled the researchers to develop a mathematically accurate method for prediction of the progression of disease by employing the integral parameter--the index of severity, as well as to reveal a number of important regularities of treatment. A follow-up of patients with reactive arthritis, Bekhterev's disease, systemic scleroderma, and other rheumatic diseases is shown to be of great importance.
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PMID:[Scientific bases of medical examinations of patients with rheumatic diseases]. 998 54

Systemic lupus erythematosus is a disease in which inflammatory process provoked by different antibodies affects many organs and systems. The circulatory system is one of them. In patients with systemic lupus erythematosus cardiac disorders are generally known and well proved. It is known that this disease has heritage background. Thus, the offsprings of patients suffering from systemic lupus erythematosus belong to a risk group. Moreover, it is thought that maternal antibodies crossing transplancentally to the fetus cause damages of tissues including the heart. The aim of this study was to evaluate cardiological status of 38 children whose mothers suffered from systemic lupus erythematosus. In all sick mothers the diagnosis fulfilled criteria of the American Rheumatism Association. The mothers have been remaining under medical treatment while the children have been under control and simultaneously prophylaxis of lupus has been undertaken. The study was undertaken in 17 girls and 21 boys aged 3 to 18 years (average: 12 +/- 4.5 years). Physical development of presented children was satisfactory. During cardiological examination all subjects were in good general condition, without any clinical evidence of collagen disease and infection. Obtained results were compared with the ones found in control group of 38 children of healthy mothers, being at the same age. Study methods were: physical examination, arterial blood pressure measurement, standard and 24 hours according to Holter method ECG record, echocardiographic and Doppler examination, and physical performance test according to Bruce's protocol. In children of sick mothers examined laboratory parameters were within the normal limits excluding the presence of antinuclear antibodies (controlled by indirect immunofluorescence test), result of which was positive in 15 studied children (39%). In the group of the children of sick mothers the abnormalities of sinus node function were detected in 12 cases (32%), significantly more often than in the control. There were found abnormalities of atrio-ventricular and intraventricular conduction in 15 subjects (40%). The premature beats of ventricular origin were noticed in 3 cases (8%). These disturbances were significantly different from the control group. In addition, correlation between the presence of antinuclear antibodies and the cardiac abnormalities was taken into consideration. So, significant correlation between antinuclear antibodies and heart rhythm disorders was proved. During echocardiographic examination structural and functional abnormalities were found. They were: ventricular septal defect (muscular part) (1), pericarditis effusion (1), prolapse of the mitral valve anterior leaflet (2), mitral valve regurgitation of the second degree (2) and increased diameter of left atrium (8). One girl from the studied group, suffering from atrio-ventricular block of III* was operated on because of persistent ductus arteriosus still in the newborn's period. At the same time the permanent pace-maker was implanted. After 1 year of age this girl was operated on because of atrial septal defect (ASD II). In studied group of children echocardiographic global indices of left ventricular systolic function were normal. The subclinical impairment of diastolic left ventricle function was found in 8 children with increased left atrium-aorta index. Both the theoretical knowledge and the results of the studies suggest that the offsprings of mothers suffering from SLE need a careful cardiological observation.
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PMID:[Cardiological state of offsprings of the mothers suffering from systemic lupus erythematosus]. 1105 11

The objective of this study was to determine the relative risks of malignancy and of site-specific malignancies in patients with systemic lupus erythematosus (SLE). A cohort of 297 patients (91% Caucasian) with SLE were seen between 1975 and 1994 and followed for a mean of 12 years at the University of Saskatchewan Rheumatic Disease Unit. Expected cancer incidence rates were determined based on Province of Saskatchewan population statistics matched to each study patient for age, sex and calendar year of follow-up. Standardized incidence ratios (SIRs) of observed to expected cancers and 95% confidence intervals (95% CI) were calculated. A total of 27 cases of cancer were observed, whereas only 16.9 were expected (SIR 1.59 (95% CI 1.05-2.32)). For site-specific malignancies, an excess of cancer of the cervix (SIR 8.15 (95% CI 1.63-23.81)) as well as hemopoietic malignancy (SIR 4.9 (95% CI 1.57-11.43)) was found. The hemopoietic cancers were predominantly non-Hodgkin's lymphoma (SIR 7.01 (95% CI 1.88-17.96)). We did not find an association of malignancy with known risk factors, including use of cytotoxic agents. Increased risk of malignancy, notably non-Hodgkin's lymphoma and perhaps cervical cancer, should be regarded as a complication of SLE.
Lupus 2001
PMID:Systemic lupus erythematosus and the risk of malignancy. 1143 73

The purpose of this study was to compare the long-term effectiveness among danazol, corticosteroids, cytotoxics, and dapsone in the treatment of hematological manifestations of systemic lupus erythematosus (SLE). Medical charts of all patients seen at the Rheumatic Disease Unit from January to December of 1998 were reviewed. Patient characteristics, disease and treatment information were collected. The main outcome measures were the cause of and time to discontinuation of drugs used to treat hematological manifestations of SLE resulting from all causes, mainly toxicity and inefficacy or both. Bivariate analysis including one-way ANOVA and chi2 tests were used to compare differences between means and proportions, respectively. Survival curves among the different drugs were evaluated using the Kaplan-Meier method. Multivariate analysis (Cox-regression) was used to adjust for potential confounders. After all medical records were reviewed 41 cases were eligible. Two cases had hemolytic anemia, 34 had thrombocytopenia, and five had both. These cases had received a total of 121 cycles of treatment at different times and they represent the study population (corticosteroids n = 37, danazol n = 51, citotoxic drugs n = 29, and dapsone n = 4). Crude rates of discontinuations due to any cause, toxicity and inefficacy werenot statistically significant among the drugs. However, the Kaplan-Meier curves showed statistically significant difference for discontinuations due to all causes as well as inefficacy. Prednisone and cytotoxic drugs had the lowest probability of continuation. In contrast, there were not statistically significant differences among the drugs with respect to first relapse. This is the first study examining the long-term termination rates of several drugs used to treat hematological manifestations of SLE. Using rates of discontinuation adjusted for time there were statistically significant differences among the drugs. Danazol had the highest probability of continuation.
Lupus 2003
PMID:Long-term effectiveness of danazol corticosteroids and cytotoxic drugs in the treatment of hematologic manifestations of systemic lupus erythematosus. 1258 27

Systemic lupus erythematosus (SLE) is an immune-mediated disease that is responsive to suppression or modulation of the immune system. Patients with SLE who experience persistent multiorgan dysfunction, despite standard doses of intravenous cyclophosphamide (Cy), represent a subset of patients at high risk of early death. We investigated the efficacy and toxicity of high-dose immunosuppression and autologous hematopoietic stem cell transplantation (SCT) to treat such patients. Six patients (all female, age 15-29 years) with severe refractory SLE were enrolled in the clinic of our institution from 1998 to 2003. All patients were seriously ill, with SLE disease activity indices (SLEDAI) of 6-30, including two cases with central nervous system lupus, one case with lung vasculitis, and three cases with nephritis and nephrotic syndrome. All patients were registered in the European Group for Blood and Marrow Transplantation (EBMT)/European League Against Rheumatism (EULAR) database. Previous immunosuppression included pulse Cy intravenous, prednisolone (standard doses and pulse therapy), oral Cy and azathioprine, with little or no effect on disease progression. Autologous hemopoietic stem cells were collected from bone marrow (n = 4) or mobilized from peripheral blood with Cy and granulocyte colony-stimulating factor (G-CSF) (n = 2). Pre-transplant conditioning regimens included BEAM +/- ATG (n = 2), melphalan 140 mg/m2 + etoposid 1600 mg/m2 (n = 2) and Cy 200 mg/kg +/- ATG (n = 2). Median time to an absolute neutrophil count (ANC) greater than 0.5 x 10(9)/L and platelet count greater than 50 x 10(9)/L was 13 and 15 days, respectively. Three patients died on days 11, 22 and 63 due to transplant-related complications. The follow-up is now 60 and six months for two patients (complete remission), and 42 months for one other patient (partial response). All patients had experienced multiple and severe episodes of infections pre-SCT and long-term history of corticosteroid therapy (3-14 years). We conclude that achievement of prolonged, corticosteroid-free remissions is a reality. Judicious selection of patients earlier in disease or in remission, but with a high risk of relapse or further progression, will diminish transplantation-related mortality.
Lupus 2004
PMID:High-dose immunosuppression with autologous stem cell transplantation in severe refractory systemic lupus erythematosus. 1499

Necrotizing histiocytic lymphadenopathy (Kikuchi's disease) is a rarely observed clinical entity characterized by fever, and solitary or multiple lymphadenopathy predominantly in the posterior cervical region. Kikuchi's disease has been reported to precede, coexist with or follow the diagnosis of systemic lupus erythematosus. In only rare instances has its association with cutaneous lupus erythematosus without systemic involvement been reported. We report a 45-year-old woman who presented characteristic systemic and cutaneous manifestations of Kikuchi's disease. Several months later, after sun exposure, she developed lesions of subacute cutaneous lupus erythematosus. The American Rheumatism Association criteria for systemic lupus erythematosus were not fulfilled. The possible pathogenic relationships between the two processes are discussed.
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PMID:Kikuchi's disease (necrotizing lymphadenitis) with cutaneous involvement associated with subacute cutaneous lupus erythematosus. 1511 1

Systemic lupus erythematosus (SLE) is responsive to treatment with immunosuppressives and steroids, but often pursues a relapsing or refractory course resulting in increasing incapacity and reduced survival. Autologous stem cell transplantation (ASCT) following immunoablative chemotherapy is a newer therapy for autoimmune disease of potential use in severe SLE. A retrospective registry survey was carried out by the European Blood and Marrow Transplant and European League Against Rheumatism (EBMT/EULAR) registry. Data was collected from 53 patients with SLE treated by ASCT in 23 centres. Disease duration before ASCT was 59 (2-155) months (median, range), 44 (83%) were female, and median age was 29 (9-52) years. At the time of ASCT a median of seven American College of Rheumatology (ACR) diagnostic criteria for SLE were present (range 2-10) and 33 (62%) had nephritis. Peripheral blood stem cells were mobilized with cyclophosphamide and granulocyte colony stimulating factor in 93% of cases. Ex vivo CD34 stem cell selection was performed in 42% of patients. Conditioning regimens employed cyclophosphamide in 84%, anti-thymocyte globulin in 76% and lymphoid irradiation in 22%. The mean duration of follow-up after ASCT was 26 (0-78) months. Remission of disease activity (SLEDAI < 3) was seen in 33/50 (66%; 95%CI 52-80) evaluable patients by six months, of which 10/31 (32%; 95%CI 15-50) subsequently relapsed after six (3-40) months. Relapse was associated with negative anti-double stranded DNA (anti-dsDNA) antibodies before ASCT (P = 0.007). There were 12 deaths after 1.5 (0-48) months, of which seven (12%; 95%CI 3-21) were related to the procedure. Mortality was associated with a longer disease course before ASCT (P = 0.036). In conclusion, this registry study demonstrates the efficacy of ASCT for remission induction of refractory SLE, although mortality appeared high. The safety of this procedure is likely to be improved by patient selection and choice of conditioning regimen. The return of disease activity in one-third of patients might be reduced by long-term immunosuppressive therapy post-ASCT.
Lupus 2004
PMID:Autologous stem cell transplantation for systemic lupus erythematosus. 1511 45

Some patients with severe systemic lupus erythematosus do not respond to conventional immunosuppression or suffer severe side effects from such treatment. In order to explore the concept of immunoablation followed by haematopoietic stem cell transplantation (HSCT) or 'rescue', an international collaboration has occurred over the past seven years. The European Group for Blood and Marrow Transplantation (EBMT) and The European League Against Rheumatism (EULAR) have analysed their collective phase I and II studies and found a remission rate (based on a reduction of the SLEDAI to < 3) in 66%, one-third of whom later relapsed to some degree. The most often used protocol was cyclophosphamide (CY) and G-CSF for mobilization and CY plus anti thymocyte globulin as conditioning. Procedure related mortality was 12% in this sick group of patients with major organ involvement. The North American, mostly single centre experience showed higher rates of remission and one procedure related death. Some relapse was also observed. Phase II studies designed to assess the role of post-HSCT maintenance therapy are being considered by the EBMT/EULAR group.
Lupus 2004
PMID:Autologous stem cell transplantation for systemic lupus erythematosus. 1523 Feb 93


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