bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cerebral magnetic resonance imaging in patients with or without antiphospholipid antibodies
Authors: Hachulla E , Michon-Pasturel U , Leys D , Pruvo J-P , Queyrel V , Masy E , Arvieux J , Caron C , Brevet-Coupeé F , Hatron P-Y , Devulder B 
Published: November 2003

Objective: To determine in patients with systemic lupus erythematosus (SLE) or with primary antiphospholipid syndrome (PAPS) the prevalence of cerebral magnetic resonance imaging changes (MRI) and the relationship with antiphospholipid antibodies. Methods: Twenty-nine consecutive SLE patients, 24 PAPS patients and 31 healthy controls were prospectively included in the study and underwent MRI Scan over a 1-year period. MRI scans were analyzed separately by a neuroradiologist for white matter changes [periventricular hyperintensity (PVH) (0–6 scale), deep white matter hyperintensity (WMH) (0–24 scale)], and one neurologist for cerebral atrophy (0–39 scale) and stroke subtypes. Statistical assessment consisted of a discriminant analysis performed with SAS-package with MRI data as dependent variables and, as independent variables, age, sex, arterial hypertension, diabetes mellitus, cardiopathy, migraine, neurological symptoms, antiphospholipid antibodies, SLE, steroid treatment. Results: The prevalence of cerebral atrophy was increased in both SLE and PAPS groups relative to controls. PVH and WMH scores were significantly higher in SLE and PAPS than in controls. Focal infarct did not differ in the SLE group when compared with PAPS. PVH and WMH scores were significantly higher in patients with neurological symptoms. Using a correlation test we found a weak significant correlation between cerebral atrophy and lupus anticoagulant. The multivariate analysis found only three independent variables related to PVH and WMH: age, the diagnosis of SLE and cerebral atrophy. Conclusions: Age, presence of SLE and presence of neurological symptoms were independently related with WMH and PVH, but not antiphospholipid antibodies.
Naturally occurring anti-idiotypic antibodies reactive with anti-DNA antibodies in systemic lupus erythematosus
Authors: Williams W M , Isenberg D A 
Published: November 2003

The levels of ‘putative’ anti-idiotypic antibodies reactive with F(ab0)2 fragments of affinity purified DNA binding antibodies from five SLE patients were measured in the serum of active and inactive systemic lupus erythematosus (SLE) patients, their relatives, spouses and healthy non-related individuals. Serum anti-idiotypic antibodies of the IgG isotype could be detected in the serum of 8=15 inactive SLE patients, 3=19 active SLE patients, 17=27 SLE relatives, 1=4 SLE spouses and 6=32 healthy non-related individuals. When the serum immunoglobulins from the individuals in each group were separated into IgG and IgM antibody fractions and analysed, a significant increase in levels of IgG anti-idiotypic reactivity were found in healthy individuals (16=32), whilst no significant increase in IgG anti-idiotypic antibodies were detected in SLE patients, SLE relatives and SLE spouses. Comparatively few individuals tested showed detectable levels of IgM anti-idiotypic antibodies. These results demonstrate that anti-idiotypic antibodies reactive with anti-DNA antibodies are detectable in the serum of SLE patients, SLE relatives, SLE spouses and healthy individuals, and are predominantly of the IgG isotype. The increased frequency of IgG anti-idiotypic antibodies after separation from serum IgM antibodies infers that anti-idiotypic activity of some IgG immunoglobulins in the sera of healthy individuals may be masked by the presence of IgM antibodies in the serum.
DNAse treatment does not improve the survival of lupus prone (NZB6NZW)F1 mice
Authors: Verthelyi D , Dybdal N , Elias K A , Klinman D M 
Published: November 2003

Objective: To examine the efficacy of deoxyribonuclease I (DNAse) therapy in the (NZB6 NZW)F1 murine model of lupus. Methods: Lupus-prone female (NZB6NZW)F1 mice were treated daily with 0–15 mg/gof recombinant DNAse for 1–6 months. Parameters including anti-DNA autoantibody production, activation of cytokine secreting cells, kidney function and longevity were monitored. Results: DNAse treatment selectively reduced the number of B cells secreting anti-dsDNA antibodies for approximately one month. However, neither short-term nor long-term treatment altered cytokine production, delayed the onset or reduced the severity of glomerulonephritis, or prolonged survival. Conclusion: DNAse treatment initiated before, during, or after the onset of murine lupus did not improve clinical outcome.
Mixed cryoglobulinaemia: a cross-road between autoimmune and lymphoproliferative disorders
Authors: Ferri C , La Civita L , Longombardo G , Zignego A L , Pasero G 
Published: November 2003

Mixed cryoglobulinaemia (MC) is a systemic vasculitis, secondary to the deposition in small and medium-sized blood vessels of circulating immune complexes, mainly the cryoglobulins, and complement. MC is characterised by a typical clinical triad (purpura, weakness, arthralgias) and by one or more organ involvement: chronic hepatitis, glomerulonephritis, peripheral neuropathy, skin ulcers and diffuse vasculitis. In a limited number of MC patients, a malignancy, that is B-cell non-Hodgkin's lymphoma or hepatocellular carcinoma, may also develop. Hepatitis C virus (HCV) infection has been found in the majority of patients with MC; the frequency of HCV markers (91%) was significantly higher than other rheumatic diseases (6.4%), namely systemic lupus, Sjùgren's syndrome, rheumatoid arthritis and systemic sclerosis, or healthy controls (1.2%). The HCV infection of lymphoid tissues may represent the remote event leading to B-lymphocyte proliferation responsible for autoantibodies and immune-complex production. In a similar way, HCV infection may also be involved in the pathogenesis of other autoimmune (glomerulonephritis, thyroiditis, lung fibrosis, autoimmune hepatitis, porphyria cutanea tarda) and lymphoproliferative disorders (monoclonal gammopathies, B-cell lymphomas). MC shares numerous clinico-serological and pathological features with the above disorders. HCV seems to be their common etiological agent; however, a variable combination of unknown co-factors (infectious, genetic, environmental) should be determinant for the appearance of different clinical patterns.
Relationship between lupus nephritis activity and the serum level of soluble VCAM-1
Authors: Ikeda Y , Fujimoto T , Ameno M , Shiiki H , Dohi K 
Published: November 2003

We measured the serum levels of soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble E-selectin (sE-selectin) and soluble intercellular adhesion molecule-1 (sICAM-1) in 72 patients with systemic lupus erythematosus (SLE) (including patients with active nephritis) and 33 normal control subjects, to investigate the correlation between levels of adhesion molecules and disease and histological activity. Serum samples were obtained at the time of renal biopsy in 27 patients with lupus nephritis. The 27 patients were divided into groups according to the World Health Organization (WHO) class as follows: class I / II, n = 11; class III / IV, n = 13 and class V, n = 3. We also determined the activity index (AI) in these 27 renal biopsy specimens. We obtained serial measurements of the serum levels of soluble adhesion molecules in 11 patients to examine the difference between active and remission stages. The serum level of sVCAM-1, but not sE-selectin or sICAM-1, was correlated with parameters of SLE disease activity, including the SLE disease activity index score, the anti-double stranded DNA antibody titer, the C3 level, the C4 level and the CH50 level. The serum levels of sVCAM-1, sE-selectin and sICAM-1 were significantly higher in patients with SLE than in controls (P = 0.006, P = 0.0005 and P = 0.04, respectively). The serum level of sVCAM-1 was significantly higher in patients with active lupus nephritis (WHO classes III and IV) than in patients in inactive lupus nephritis (WHO classes I and II) (P = 0.0016). The sVCAM-1 level was significantly elevated in patients with an AI > 4 compared with patients with an AI < 4(P = 0.0025). The sVCAM-1 level decreased significantly during remission (P = 0.0033). The serum level of sVCAM-1 was elevated in patients with active lupus nephritis (WHO classes III and IV) and in patients with high AI scores. The serum level of sVCAM-1 was correlated with the SLE disease activity and decreased during remission. Therefore, the sVCAM-1 level may be a useful marker of lupus nephritis activity.
Plasma homovanillic acid and prolactin in systemic lupus erythematosus
Authors: Ferreira C , Paes M , Gouveia A , Ferreira E , Pádua F , Fiuáza T 
Published: November 2003

Prolactin (PRL) is an important immunoregulator and might have a role in the pathogenesis of systemic lupus erythematosus (SLE). The regulation of pituitary prolactin secretion is complex and involves a negative feedback process in the hypothalamus, in which dopamine plays the principal role. However, the main source of serum prolactin in lupus patients is still not clearly established. Since homovanillic acid (HVA), the principal metabolite of dopamine (DA), is removed from the brain into the blood, it would indirectly reflect DA metabolism. It is assumed that the turnover of a neurotransmitter can be determined through an analysis of its metabolites. The objective of this study was to analyse plasma samples from SLE patients to see if there were any alterations in neurally functioning DA through its principal metabolite, HVA. We also measured the levels of PRL and compared HVA and PRL with the clinical activity of the disease. Twenty-four SLE patients and fifteen healthy controls were studied. The investigation was done over a period of 3 months. The results of this study show significantly low levels of HVA in lupus patients compared to controls (P < 0.0001). This corresponds to a decrease in dopamine turnover. Hyperprolactinemia was observed in nine patients, and the average level of prolactin in lupus patients was higher than in healthy controls (P < 0.001). For the duration of the study, a significant percentage of variation was observed in the levels of HVA in the clinically active patients (P < 0.05) compared to inactive patients. When PRL was compared in these groups, throughout the study, no signifcant percentage of variation was observed. The relationship between HVA and PRL in healthy controls was r = 0.47, P=0.08, and in patients was r = 0.04, P = 0.84. It is suggested that there is a probable association between plasma levels of HVA and PRL in the healthy controls and not in the SLE patients.
Bromocriptine in systemic lupus erythematosus: a double-blind, randomized, placebo-controlled study
Authors: Alvarez-Nemegyei J , Cobarrubias-Cobos A , Escalante-Triay F , Sosa-Muüoz J , Miranda J M , Jara L J 
Published: November 2003

The objective of this study was to investigate the efficacy and safety of bromocriptine (BRC) as an adjunct to conventional treatment in systemic lupus erythematosus (SLE). A prospective, doubleblind, randomized, placebo-controlled study compared BRC at a fixed daily dosage of 2.5 mg with placebo. Patients were followed for 2–17 months (mean 12.5 months). Disease activity was assessed using the SLE Disease Activity Index (SLEDAI), numbers of flares were recorded, and serum prolactin (PRL) levels were obtained at intervals during the study. Patients were allowed to take prednisone and immunosuppressive drugs. Sixty-six patients with SLE entered the study. Thirty-six were treated with BRC, and 30 controls received placebo. Sixteen patients were removed from the study during the treatment period: five in each group left the study because of adverse effects, five became pregnant, and one patient who took placebo died with central nervous system lupus. Four patients in the BRC treatment group and three patients in the placebo group moved away or stopped coming for study visits for unknown reasons, and were lost to follow-up during the course. At entry, serum PRL was (mean–s.d.) 24.8 ng/ml–18.4 in the BRC treatment group. This value fell to 5.8–9.0 after 12 months of treatment. Corresponding PRL values in controls were 23.7–22.1 pretreatment and 20.3–14 after 12 months. PRL levels in BRC-treated subjects were significantly lower than levels in control subjects after 3, 6, 9, and 12 months of treatment. The SLEDAI score on the fifth protocol visit was decreased significantly in the BRC group vs controls: 0.9–1.4 vs 2.6–4.5 (P < 0.05). Although the absolute number of flares in each group was similar, the mean number of flares/patient/month was decreased significantly in the BRC group compared to the control group (0.08–0.1 vs 0.18–0.2, P = 0.03). Long term treatment with a low dose of BRC appears to be a safe and effective means of decreasing SLE flares in SLE patients.
Hypothalamic-pituitary-adrenal axis function and prolactin secretion in systemic lupus erythematosus
Authors: Gutiérrez M A , Garcia M E , Rodriguez J A , Rivero S , Jacobelli S 
Published: November 2003

The objective was to study the response of cortisol and of prolactin (PRL) to specific stimuli in systemic lupus erythematosus (SLE). We measured the response of cortisol to insulin-induced hypoglycemia and of PRL to thyrotropin releasing hormone (TRH) in seven patients with active untreated SLE and in ten paired control subjects. All were women with regular menstrual cycles. With the exception of two patients, they had never received corticosteroids before the study. The basal serum levels of cortisol (12.5 2.4 μg/dl) and PRL (10.7 1.0 ng/ml) in the SLE group were not significantly different from those of the control group (12.3 1.1 mg/dl and 13.7 2.4 ng =ml, respectively). The cortisol response after hypoglycemia was significantly lower in SLE patients compared to the control group at 45 min (P = 0.01), at 60 min (P = 0.009), and at 90 min (P = 0.001). The integrated cortisol response to hypoglycemia expressed as area under the response curve (AUC) did not differ significantly in either group (1447 187 vs 1828 84, P = 0.06). Although the peak of PRL after TRH did not differ significantly in both groups (68.0 7.4 ng =ml in SLE vs 66.3 77 ng/ml in controls) and the AUC of PRL response after TRH was comparable in both groups (4672 537 vs 4128 541, P = 0.32), the interval-specific ‘delta’ response was significantly higher in SLE than the control group at 0–60 min (P = 0.02) and 0–90 min (P = 0.01) after TRH injection. These findings suggest that active SLE is associated with some degree of dysfunction of the hypothalamic-pituitary-glucocorticoid axis and PRL secretion.
Autologous peripheral blood stem and progenitor (CD34+) cell transplantation for systemic lupus erythematosus complicated by Evans Syndrome
Authors: Musso M , Porretto F , Crescimanno A , Bondi F , Polizzi V , Scalone R , Mariani G 
Published: November 2003

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 encouraging 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 lowtitre 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.
Vascular complications of diabetes mellitus: what role for phospholipid-binding antibodies?
Authors: Galtier-Dereure F , Biron C , Vies M , Bourgeois V , Schved J-F , Bringer J 
Published: November 2003

Vascular complications are the main cause of morbidity in diabetes mellitus. However, the risk factors for vascular disease remain uncompletely elucidated. It has been previously suggested that factors other than glycemia may contribute to the development of vasculopathy. In this study we determined the prevalence of phospholipid-binding antibodies in uncomplicated and complicated diabetes. We studied 53 uncomplicated diabetic patients, with type 1 (n=32) or type 2 (n=21) diabetes; 23 diabetic patients with proliferative retinopathy; 28 diabetic patients with an overt nephropathy; 37 diabetic patients with macroangiopathy and 22 non diabetic control patients. Both lupus anticoagulant and anticardiolipin antibodies were determined.Other risk factors for macroangiopathy were analysed. The prevalence of phospholipid-binding antibodies was similar in uncomplicated diabetic patients and in controls (type 1 diabetes: 9.4&percnt;; type 2 diabetes: 9.5&percnt;; control group: 4.6&percnt;; P=0.76). In complicated diabetes, the frequency of these antibodies was increased only in patients with overt nephropathy (32.1&percnt;, P=0.01) or with macroangiopathy (32.4&percnt;, P=0.01) while patients with isolated retinopathy were comparable with uncomplicated diabetic patients (4.3&percnt;, P=0.66).Uncomplicated diabetes was not associated with phospholipid-binding antibodies. We found a higher prevalence of these antibodies in diabetic patients with macroangiopathy or nephropathy. These results suggest a potential role of phospholipid-binding antibodies in the progression of vascular complications in diabetes mellitus.
Echocardiography in systemic lupus erythematosus
Authors: Kalke S , Balakrishanan C , Mangat G , Mittal G , Kumar N , Joshi V R 
Published: November 2003

Two dimensional echocardiography with doppler examination was performed in 54 patients with systemic lupus erythematosus (SLE). Nine (17%) had significant cardiac involvement (four left ventricular hypertrophy, one moderate pericardial effusion, one severe aortic regurgitation, and three ventricular systolic dysfunction). We further studied diastolic function in 45 patients who did not have a major abnormality in echo. SLE was graded as active in 16 patients (SLEDAI > 5) and inactive in 29 patients. Twenty age-and sex-matched subjects acted as controls. The data were compared using one way ANOVA test. Patients with active disease had significant diastolic dysfunction compared to inactive patients and controls as indicated by increased peak A (P < 0.01) and decreased E/A ratio (P < 0.01). There was no linear correlation between disease activity and diastolic dysfunction if SLEDAI was considered as a continuous variable (r = 0.29 for E =A). Anticardiolipin antibodies (both IgG and IgM) were elevated in five patients (13 studied). One of them had severe mitral regurgitation, one had trace mitral and aortic regurgitation and one had diastolic dysfunction. We conclude that asymptomatic diastolic dysfunction is present in SLE patients.
Anticardiolipin antibodies in childhood rheumatic disorders
Authors: Gedalia A , Molina J F , García C O , Doggett S , Espinoza L R , Gharavi A E 
Published: November 2003

Anticardiolipin antibodies (aCL) have been reported to occur in a wide variety of autoimmune and non-autoimmune disorders in adults. Our objective was to investigate the prevalence and isotype distribution of aCL and its relationship with the features of antiphospholipid syndrome (APS) in childhood rheumatic disorders. Between November 1995 and May 1996, all patients who visited our paediatric rheumatology clinic whose guardians signed a consent form participated in the study. The study population included 106 patients (36 systemic lupus erythematosus (SLE), 28 juvenile rheumatoid arthritis (JRA), 11 fibromyalgia, 7 sarcoidosis, 5 dermatomyositis, 3 rheumatic fever (RF), 3 vasculitis, 2 scleroderma, and 11 miscellaneous). aCL measurements were performed by enzyme linked immunoabsorbent assay (ELISA). All patients were carefully evaluated for symptoms and signs of APS. Eighteen of the 106 patients (17%) were tested positive for one or more of the three aCL isotypes. In SLE, aCL were found positive in 13 of 36 (37%); in JRA 2 of 28 (7%); in sarcoidosis 2 of 7; and in RF 1 of 3. aCL of IgG isotype were found positive in 16 patients (11 SLE, 2 sarcoidosis, 2 JRA, and 1 RF). This isotype was usually detected at low titers (16–24 GPL). aCL of IgM isotype were found positive in five patients (2 sarcoidosis, 2 SLE, 1 JRA), and aCL of IgA isotype were found positive in only three patients (2 SLE, 1 sarcoidosis). Clinical features of APS were rarely seen in our SLE population and were not associated with the presence of aCL. None of the patients in the other groups exhibited any clinical manifestations of APS. In conclusion, aCL were found in 37% of our childhood SLE patients as compared with only 7% in JRA. These were mostly aCL of IgG isotype of low titers and therefore were not associated with the main features of APS. Prospective studies with a larger sample size may be needed to ascertain the exact prevalence and clinical significance of aCL in childhood-onset SLE.
Lymphocyte apoptosis in systemic lupus erythematosus: relationships with Fas expression, serum soluble Fas and disease activity
Authors: Courtney P A , Crockard A D , Williamson K , McConnell J , Kennedy R J , Bell A L 
Published: November 2003

Lupus specific autoantigens are exposed on apoptotic cells. The increased number of apoptotic lymphocytes reported in systemic lupus erythematosus (SLE) may be attributable to abnormalities of lymphocyte Fas expression or serum soluble Fas. In the present study we analysed the count of circulating apoptotic lymphocytes in SLE patients (n ‘ 50), by flow cytometry using Annexin V, compared to rheumatoid arthritis patients (RA, n ‘ 20), inflammatory bowel disease patients (IBD, n ‘ 20) and normal controls (n ‘ 20). Lymphocyte Fas expression and serum soluble Fas were measured and related to numbers of apoptotic lymphocytes. The percentage of apoptotic lymphocytes, determined by Annexin V binding, was significantly increased in peripheral blood of SLE patients (median ‘ 4.2%) compared with normal healthy donors (median ‘ 1.1%) and IBD patients (median ‘ 2.0%) but not RA (median ‘ 3.9%). SLE lymphocyte Fas expression was not significantly different from RA or IBD patients. Serum soluble Fas in SLE patients correlated positively with apoptotic lymphocytes and antibodies to double stranded DNA. This study suggests that increased apoptotic lymphocytes and increased lymphocyte Fas expression may not be specific to SLE. Serum soluble Fas may have a role in the regulation of lymphocyte apoptosis in SLE.