bluuebunny/crossref_metadata_2025_split huggingface dataset:
Vascular events in hypertensive patients with systemic lupus erythematosus
Authors: Rahman P , Aguero S , Gladman D D , Hallett D , Urowitz M B 
Published: November 2003

Our purpose was to examine prospectively the relationship between systemic hypertension and vascular events in patients with SLE. SLE patients followed in the University of Toronto Lupus Clinic presenting between 1980 and 1988 and within one year of their diagnosis of SLE were identified. Standard definitions were used for hypertension and for all vascular events (MI, angina, CVA, PVD). The presence of traditional CAD risk factors, along with disease- and therapy-related risk factors for the development of vascular disease, were compared in the hypertensive and normotensive group. A multivariate logistic regression was performed to determine the best predictor of a vascular event. One hundred and fifty patients were identified in our inception cohort [75 hypertensive (50%) and 75 (50%) normotensive]. Seventeen hypertensive patients (22.7%) had at least one vascular event as compared to six (8.0%) normotensive patients (P = 0.022). The vascular events included 7 with CAD, 5 with CVA, and 5 with PVD in the hypertensive group while in the normotensive group 3 patients developed CAD, 2 CVA and 1 PVD. Fifteen deaths were recorded in the hypertensive group as compared to eight deaths in the non-hypertensive groups (P = 0.09). The groups were comparable with respect to associated risk factors, except for higher frequency of hypercholesterolemia (P = 0.003), azotemia (P = 0.001) and corticosteroid use (P = 0.038) in the hypertension group. In a multivariate analysis the best predictor of a vascular event was hypercholesterolemia (OR 6.9, 95% CI 2.4–24.8, P < 0.001). We conclude that systemic hypertension is associated with an increased frequency of vascular events in SLE. This is best explained by its association with hypercholesterolemia.
Safety profile and causes of withdrawal due to adverse events in systemic lupus erythematosus patients treated long-term with cyclosporine A
Authors: Conti F , Priori R , Alessandri C , Spinelli F R , Medda E , Valesini G 
Published: November 2003

Several case reports and uncontrolled trials have established the use of cyclosporine A (CsA) in systemic lupus erythematosus (SLE) but some concerns have been raised because of its kidney damaging effects. We here report the results of a retrospective follow-up study designed to assess the safety profile and causes of discontinuation due to adverse events in SLE patients treated with CsA. We treated 56 SLE patients with oral CsA at doses of 3 ± 5 mg/kg for an average of 26 months. Adverse events not leading to the discontinuation of therapy were observed in 62.5% of the patients, the most frequent being hypertrichosis. CsA was stopped because of the occurrence of side effects in 9=56 (16%) of the patients. The most common were nephrotoxicity (3=9) and the occurrence of tremors (3=9). These effects were always reversible within three months of CsA withdrawal. The patients who were older than 40 y had a significant slightly increased risk of stopping CsA therapy for any adverse events (RR 1.08; CI 95% 1.03 ± 1.14). In comparison with previous studies, this study involved a larger cohort of SLE patients who were evaluated for a longer period of follow-up, and confirmed the good tolerability of CsA in these subjects.
Systemic lupus erythematosus presenting with a reticular erythematous mucinosis-like condition
Authors: Pozo J Del , Peña C , Almagro M , Yebra M T , Martínez W 
Published: November 2003

We report the case of a woman with clinical and histopathological features of reticular erythematous mucinosis syndrome (REM) who six years later developed diagnostic criteria for systemic lupus erythematosus (SLE). Two biopsies performed at the same area initially showed mucin dermal deposits and further development of characteristic dermo-epidermal changes of lupus erythematosus. Our findings suggest that SLE may present with a REM-like condition.
ANA negative systemic lupus erythematosus sera revisited serologically
Authors: Reichlin Morris 
Published: November 2003

Objective: To better define the serology of a panel of sera from patients with a clinical diagnosis of systemic lupus erythematosus (SLE) or subacute cutaneous lupus erythematosus (SCLE) with a negative antinuclear antibody (ANA) test on mouse liver. Methods: Sensitive ELISA methods for anti-Ro/SSA, anti-La/SSB, and anti-U1RNP were applied to a panel of 76 sera with either SLE or SCLE and a negative ANA test on mouse liver. Results: These sera had previously been shown to have a high prevalence of anti-Ro/SSA (68%) and anti-La/SSB (27%) precipitins respectively. None had precipitins to U1RNP or Sm. ELISA methodology revealed that all of the sera 76=76 (100%) had elevated levels (Mean±2SDofa panel of 21 normal sera) of anti-Ro/SSA, 36=76 (46%) had elevated levels of anti-La/SSB, and 27 of 76 (35%) had elevated levels of anti-U1RNP. Conclusion: The subset of patients with SLE and SCLE with a negative ANA test on mouse liver almost uniformly have antibodies to the Ro/SSA antigen by a sensitive ELISA. This adds evidence to the idea that this is a more homogeneous disease subset within the spectrum of SLE.
Serum levels of soluble Fas correlate with indices of organ damage in systemic lupus erythematosus
Authors: Al-Maini Mustafa H , Mountz John D , Al-Mohri Huda A , El-Ageb Elnour M , Al-Riyami Bazdawi M , Svenson Karin L G , Zhou Tong , Richens Elizabeth R 
Published: November 2003

Objective: To evaluate whether the levels of soluble form of the Fas apoptosis antigen (sCD95=sFas) varied from those of healthy control subjects in a group of patients with systemic lupus erythematosus (SLE). This was done to determine whether sFas has a role in either the disease activity or the organ damage in SLE. Methods: Serum levels of sFas were measured over a period of 4 y (277 determinations) in 39 Arab patients with SLE and 22 age-, gender-, and race-matched healthy controls using double antibody ELISA. SLEDAI scores for disease activity and SLICC/ACR scores for cumulative organ damage were determined. Serum levels of acute phase reactants, complement, inflammatory cell counts, levels of autoantibodies, and kidney and liver function test results were obtained retrospectively from clinical records. Results: sFas levels were significantly higher in patients with SLE (n = 39, 277 determinations) (0.60 ng/ml± 0.38) than in healthy controls (n = 22) (0.26 ng/ml± 0.11) (P < 0.00001). The levels of sFas correlated with SLICC/ACR (r = 0.36; P < 0.02), but not with SLEDAI. sFas correlated with renal and liver function tests measured by s-creatinine (r = 0.38; P:30, P < 0.001), s-albumin (r =-0:28, P < 0.0001), and ALT (r = 0.35; P < 0.00001), but did not correlate with the levels of acute phase reactants. Conclusion: sFas is elevated in sera of SLE patient. Since sFas correlates with indices of organ damage but not with disease activity, it may be a marker of organ damage in SLE and may act to protect certain organs from further damage by inhibiting Fas-mediated apoptosis.
Autoimmunity in atherosclerosis: lessons from experimental models
Authors: George J , Afek A , Gilburd B , Harats D , Shoenfeld Y 
Published: November 2003

The modern view of atherosclerosis is of a chronic inflammatory disorder. In accord with this paradigm, the process of uninhibited influx of fat to the vessel wall results from an ‘adequate’ response to various forms of injury (i.e. turbulence, infections, modified lipoproteins). This idea has been further extended by several groups, to assume that the atherosclerotic lesion can be the target of an autoimmune mediated attack. According to this hypothesis, the site of initiation of the plaque should bear/express the target autoantigen, whereas concomitantly a respective immune response is generated in the periphery. The examples illuminating this notion are β2GPI as a target autoantigen, HSP60/65 an oxidized-LDL. Herein we present evidence to support the involvement of autoimmune mechanisms in atherogenesis based on the experience from experimental models and human studies.
Lipoprotein(a) oxidation and autoantibodies: a new path in atherothrombosis
Authors: Romero F I , Khamashta M A , Hughes G RV 
Published: November 2003

Lipoprotein(a) (Lp(a)) is considered a vascular pathogen of outstanding importance. High plasma levels of this lipoprotein are associated with premature arterial disease; however, the mechanisms involved have not been clarified. The atherosclerotic process is increasingly regarded as a chronic inflammatory reaction in the arterial wall where oxidation-mediated endothelial injury involving modified forms of low-density lipoprotein (LDL) seems to be a key event. Autoimmune pathways are involved in the progression of atherosclerosis and humoral response to oxidatively modified LDL can be considered among these pathways. A number of factors can be encountered in the pathogenesis of the accelerated arterial disease seen in patients with antiphospholipid (Hughes) syndrome (APS) and systemic lupus erythematosus (SLE). Among these, high levels of Lp(a) have been described in both and increasing evidence indicates that patients with antiphospholipid antibodies (aPL) are under oxidative stress. Recent studies suggest that the so-called ‘oxidation theory of atherosclerosis’ may also be applied to Lp(a). This fact makes this lipoprotein potentially suitable as a target of the immune system and antibodies reacting against oxidatively-modified Lp(a) by malondialdehyde have been recently described in APS and SLE. It is therefore likely that an immune response to the oxidized moiety of Lp(a) might be influential in the pathogenicity of this lipoprotein and, subsequently, of atherosclerosis.
Lost-to-follow-up study in systemic lupus erythematosus (SLE)
Authors: Gladman D D , Koh D-R , Urowitz M B , Farewell V T 
Published: November 2003

Objective: To determine the extent of and reasons for lost-to-follow-up, as well as its impact on outcome studies in a cohort of lupus patients. Methods: As of September 1991, 247 patients, in a cohort of 621 patients with SLE, being followed in a long-term prognosis study, had not been seen since 1 March, 1990 and were considered lost-to-follow-up. Patients were contacted and encouraged to return for an evaluation or to answer a questionnaire by telephone. Descriptive statistics were used to compare the lost-to-follow-up and non-lost-to-follow-up patients and the survival experience during the lost-to-follow-up period was compared with that when patients were not considered lost-to-follow-up. Estimated survival curves with and without the information gained through contacts with lost-to-follow-up patients were compared. Results: Of the 247 patients, 29 have died, 66 returned for a full assessment, 84 completed a questionnaire and 68 (11%) were true lost-to-follow-up. The lost-to-follow-up patient group had 10% more Caucasians and 6% more males than the patients under regular follow-up. The estimated survival curves of the entire cohort with and without the new lost-to-follow-up data, calculated as of July 1992, were very similar. There was no evidence of a differential mortality rate during the period in which patients were lost-to-follow-up. Some suggestive evidence that the relative mortality rate comparing the rate during a period of lost-to-follow-up and during a period of active follow-up may depend on disease duration at the time of lost-to-follow-up was found. Conclusions: While it would be prudent to limit lost-to-follow-up as much as possible, especially for outcomes such as mortality which do not necessarily require a clinic visit, it does not appear that significant bias will be present in prospective studies based on our single clinic database. Since the retrieved 179 lost-to-follow-up patients did not affect survival studies it is likely that the 68 true lost-to-follow-up patients will also not have an impact on prognostic studies.
Accuracy of medical record scoring of the SLICC/ACR Damage Index for systemic lupus erythematosus
Authors: Thumboo J , Lee H-Y , Fong K-Y , Chan S-P , Chapman CAT , Leong K-H , Feng P-H , Thio S-T , Boey M-L 
Published: November 2003

Objective: To study the accuracy of medical record scoring of the SLICC/ACR Damage Index (DI) for SLE. Methods: Medical record DI (MDI) scoring of 60 SLE patients by one physician was compared with prospective direct DI (DDI) scoring a median of 2 y earlier. Agreement between DDI and MDI total and organ system scores was compared using Cohen's kappa, the degree of misclassification by MDI and the significance of differences between DDI and MDI scores. Results: The mean duration of SLE at DDI scoring was 5.2 y. The median/mean (SD) DDI and MDI total scores were 0=0.78 (1.38) and 0=0.85 (1.44). Damage was present on DDI and MDI scoring in 36.7% (22=60) and 38.3% (23=60) of patients. Qualitative MDI and DDI total scores (damage present/absent) showed good agreement (kappa ‘ 0.61, 95% CI ‘ 0.40–0.82, 18.3% misclassification). Quantitative MDI and DDI total scores (degree of damage) showed moderate agreement (kappa ‘ 0.47, 95% CI ‘ 0.28–0.66, 36.7% misclassification). 9 of 12 MDI organ system scores misclassified < 10% of subjects. There was no statistically significant difference between DDI and MDI total or organ system scores. Conclusion: MDI total scoring is a good qualitative and moderate quantitative reflection of SLE related damage. MDI organ system scores are more accurate than MDI total scores.
Evaluation of cardiac abnormalities and embolic sources in primary antiphospholipid syndrome by transesophageal echocardiography
Authors: Turiel M , Muzzupappa S , Gottardi B , Crema C , Sarzi-Puttini P , Rossi E 
Published: November 2003

Objective: Valvular lesions are frequently present in Primary Antiphospholipid Syndrome (PAPS) patients using transthoracic and/or transesophageal echocardiography. The aim of this study was to describe the prevalence of cardiac abnormalities (valvular thickening and/or regurgitation) or potential embolic sources (spontaneous echocontrast and/or vegetations) in PAPS patients. Methods: Multiplane transesophageal echocardiography was performed consecutively on 40 PAPS patients: 17 of them with thrombocytopenia, 27 with at least one thromboembolic event (stroke, transient ischaemic attack, arterial and/or venous thrombosis, pulmonary embolism) and 14 with recurrent fetal loss. Design: Cardiac involvement (cardiac abnormalities and/or embolic sources) was present in 33/40 (82%) of PAPS patients. According to aCL titer these lesions were revealed in 17/24 (71%) of patients with aCL < 40 GPL-U, while these lesions were present in 100% of patients with aCL > 40 GPL-U. Three patients presented mitral stenosis and 3 non-infective valve masses or vegetations. Embolic sources were found in 4/24 (17%) patients with aCL <40 GPL-U, while they were observed in 6/16 (37%) of patients with titer of aCL >40 GPL-U (X2- 10.03, P <0.01). Regression analysis showed a positive correlation between mitral valve thickening and aCL antibodies titer (r= 0.5; P<0.001). Conclusions: Valvular lesions are commonly found in PAPS patients. Our data showed a significant correlation among aCL titer, mitral leaflets thickening and thromboembolic events.
A novel treatment for lupus nephritis: lignan precursor derived from flax
Authors: Clark W F , Muir A D , Westcott N D , Parbtani A 
Published: November 2003

Background: Flaxseed has renoprotective effects in animal and human lupus nephritis. We have recently extracted the lignan precursor (secoisolariresinol diglucoside) (SDG) to determine if this more palatable derivative of flaxseed would exert renoprotection similar to the whole flaxseed in the aggressive MRL/lpr lupus mouse model. Methods: 131 MRL/lpr mice were randomly assigned to saline gavage, 600, 1200 and 4800 μg lignan gavage groups. At 7 weeks, 6 animals underwent platelet aggregating factor (PAF) lethal challenge and 40 were studied with urine collection to determine the levels of secoisolariresinol, enterodiol and enterolactone in the gavaged animals. A baseline study of 10 saline gavaged animals took place at 6 weeks. 25 animals in the saline gavage, 600 and 1200 μg lignan groups were studied at 14 and 22 weeks for GFR, spleen lymphocyte S-phase and organ weight studies. Results: Metabolic studies indicated that secoisolariresinol is the major metabolite absorbed and the lowest lignan dose provides a lengthening in survival for the PAF lethal challenge. Body weight, fluid and water intake studies demonstrated that the lignan was well tolerated. Changes in proteinuria, GFR and renal size showed a time-and dose-dependent protection for the lignan precursor. Cervical lymph node size and spleen lymphocyte cells in the S-phase demonstrated modest dose-dependent reductions in the lignan gavaged groups. Conclusion: SDG was converted in the gut to secoisolariresinol, which was absorbed and well tolerated by the MRL/lpr mice. Renoprotection was evidenced, in a dose-dependent fashion, by a significant delay in the onset of proteinuria with preservation in GFR and renal size. This study suggests that SDG may have a therapeutic role in lupus nephritis.
The validity of the ECLAM index for the retrospective evaluation of disease activity in systemic lupus erythematosus
Authors: Mosca M , Bencivelli W , Vitali C , Carrai P , Neri R , Bombardieri S 
Published: November 2003

Aim: To determine whether the European Consensus Lupus Activity Measurement Index (ECLAM) can be used to evaluate disease activity in patients retrospectively from the data provided in their clinical charts. Methods: The ECLAM score was calculated twice in a series of 64 consecutive SLE patients: first for each patient during the course of a standard clinical evaluation (direct-ECLAM), and then one to two weeks later solely on the basis of the data provided in the patient's clinical chart (chart-ECLAM). The scorings for each patient were performed by two different assessors. Results: The direct-ECLAM and chart-ECLAM scores were highly correlated (Spearman's rank correlation coefficient = 0.86). The regression line was not significantly different from the identity line (t-test). The Pearson's coefficient was 0.88. The interobserver variability of the chart-ECLAM showed a low inter-rater variability. Conclusion: ECLAM could represent a valid and reliable instrument for the retrospective analysis of disease activity in SLE patients.
Antiphospholipid antibodies in alcoholic liver disease are influenced by histological damage but not by alcohol consumption
Authors: Perney P , Biron-Andreani C , Joomaye Z , Fabbro-Peray P , Quenet F , Schved J F , Blanc F 
Published: November 2003

Antiphospholipid antibodies (aPL) have been detected in various liver diseases, particularly cirrhosis. The role of alcoholic consumption per se has been suggested. The aim of our study was to assess the prevalence of aPL in patients with alcoholic liver disease at various states and to correlate the presence of aPL with both liver injury and alcoholic consumption. Three groups were prospectively included. Group A: 74 controls (age-and sex-matched); group B: 46 patients with alcoholic steatosis; group C: 28 patients with alcoholic cirrhosis. For each patient, lupus anticoagulant, anticardiolipin antibodies and anti-j32-glycoprotein I antibodies were tested. The prevalence of aPL (presence of at least one positive test) was 5% in group A, 20% in group B and 50% in group C (P < 0.04). No correlation appeared between aPL and Child Pugh score in group C. No correlation was found between the presence of aPL and alcohol intake in patients with either steatosis or cirrhosis. Our study confirms that aPL positivity is more frequently encountered in patients with alcoholic liver disease than in controls. Their prevalence increases with the degree of histological damage but not with the level of alcoholic intake.
Psychiatric and psychosocial disorders in patients with systemic lupus erythematosus: a longitudinal study of active and inactive stages of the disease
Authors: Seguí J , Ramos-Casals M , García-Carrasco M , de Flores T , Cervera R , Valdeés M , Font J , Ingelmo M 
Published: November 2003

The objective was to analyze psychiatric disorders and psychosocial dysfunction in patients with systemic lupus erythematosus (SLE), studied longitudinally during active and subsequent inactive stage of their disease. During a 6 month period of study, we selected 20 consecutive patients with SLE who presented with a SLE flare. All patients fulfilled the 1982 revised criteria of the American College of Rheumatology for the classification of SLE. When patients entered the study, we performed psychiatric (CIS, RDC, STAI, HD, BDI, GHQ and MMS) psychosocial (GAS and VAS-P) scores assessment. One year later, we repeated the psychiatric and psychosocial assessment when patients showed inactive disease. The 20 patients evaluated were women, with a mean age of 34 y (SE 14.4, range 20-57). According to CIS evaluation, we diagnosed 8 (40%) psychiatric cases in the acute episode of SLE. The RDC diagnosis showed generalized anxiety in 5 patients, panic disorders in 2 patients and generalized anxiety plus depressive symptoms in one patient. One year later, when patients did not show disease activity, we diagnosed 2 (10%) psychiatric cases (P < 0.05). When SLE patients were clinically inactive, they showed lower levels of psychological distress (GHQ scale, 1.8 vs 5.6, P < 0.001), with a lower grade of anxiety measured by both HA (3.2 vs 8.2, P < 0.01) and STAI-S (7.95 vs 20.90, P < 0.001) scales. We also found a lower score in pain perception (VAS-P) (2.80 vs 4.25, P < 0.01) and higher occupational activity (VAS-P) (83.9 vs 66.2, P < 0.01) and general functioning (GAS) (93.75 vs 83.50, P < 0.05) during the inactive stage. No significant differences were found when we compared cognitive impairment, grade of depression and physical disability between inactive and active stages. We conclude that in SLE patients, psychiatric and psychosocial disorders during acute episodes are usually mild and seem to be related to the psychological impact of disease activity on patients. This type of psychiatric pathology is similar to that which would be expected in other groups coping with a stressful event, indicating that our patients did not react in a way specifically determined by their systemic disease.
Increased serum soluble CD14, ICAM-1 and E-selectin correlate with disease activity and prognosis in systemic lupus erythematosus
Authors: Egerer K , Feist E , Rohr U , Pruss A , Burmester G-R , Dörner T 
Published: November 2003

To improve monitoring of immunological and disease activity, we determined soluble markers of activity of the monocyte/macrophage system (sCD14) and the vascular endothelium (sE-selectin, sICAM-1) in patients with systemic lupus erythematosus (SLE) and primary Sjögren' syndrome (pSS) in comparison to patients with infections or sepsis. Concentrations of sCD14, sICAM-1 and sE-selectin (soluble CD14, ICAM-1 and E-selectin, respectively) were measured in serum samples from patients with SLE and pSS, patients with sepsis, different infectious diseases and healthy controls using ELISA systems. Elevated levels of sE-selectin and sICAM-1 were detected in patients with SLE as well as sepsis, in contrast to patients with a localized infection (SLE and sepsis, respectively, versus infection P < 0.001; Kruskal–Wallis test). Levels of sCD14 were persistently elevated in sera from patients with SLE, whereas these values decreased rapidly after effective therapy in patients with sepsis or infection. A continuous elevation of all of these three parameters was associated with a fatal outcome in patients with sepsis as well as in patients with SLE. Combined elevation of sCD14, sICAM-1 and sE-selectin correlates with the prognosis in patients with active SLE and indicates a remarkable immune activation involving the monocyte/macrophage system and the endothelium comparable to an activation found only in patients with sepsis.