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Objectives: To clarify clinical manifestations, association with disease activity, and prognostic impact of thrombocytopenia using simple and reliable indices. Methods: 632 patients were reviewed retrospectively. Fifty patients with thrombocytopenia were included as cases and matched with 100 control patients. Clinical manifestations at first thrombocytopenic episode were recorded. Classification criteria at diagnosis, basic immunological profiles, disease activity (ECLAM), and end organ damage (SLICC) were recorded. Results: 29/50 (58%) had thrombocytopenia at diagnosis of lupus. Haemorrhagic manifestations were associated with the degree of thrombocytopenia (p<0.001). Anticardiolipin antibodies were not related to the degree of thrombocytopenia or the severity of haemorrhagic manifestations. Megakaryocytes were normal or increased in 26/28 (93%) bone marrow specimens, indicating peripheral platelet destruction. Patients with high disease activity were more thrombocytopenic than controls (OR = 2.61, 95% CI 1.13 to 5.96, p = 0.009). Patients with low C3 or CH50 were more likely to be thrombocytopenic (OR = 2.36, 95% CI 1.05 to 5.26, p = 0.029). Median SLICC for lupus patients with thrombocytopenia was 2 (range 0–11) compared with 1 (range 0–12) for controls (p<0.001). No deaths occurred during thrombocytopenic episodes. Conclusions: Thrombocytopenia is not directly associated with end organ damage and mortality, but defines a subgroup of patients with higher morbidity and is thus a major complication of systemic lupus erythematosus, affecting overall prognosis.

Background: Joint involvement occurs in most patients with systemic lupus erythematosus (SLE), and severe lupus arthritis is often refractory to conventional treatments. Anakinra is used in the treatment of rheumatoid arthritis, but its therapeutic potential has not been proved in patients with SLE. Objective: To determine the safety/tolerability and efficacy of anakinra in patients with SLE with leading joint involvement. Methods: In patients with SLE with active polyarthritis and no other uncontrolled systemic/organ manifestations, 100 mg/day anakinra was self administered subcutaneously for 3 months. Disease activity was assessed by VAS, number of swollen/tender joints, ECLAM score, and serological and immunological measures. Results: Four patients with SLE were studied; anakinra was safe in all four patients and no drug related serious adverse events occurred. A subjective benefit was seen in all patients and a trend towards better activity measures after 4 weeks. After an initial response, one patient left the study because of an arthritic flare after 6 weeks. Conclusion: In this study anakinra was apparently safe and well tolerated and led to clinical and serological improvement. Anakinra might be an interesting alternative in individual patients with lupus arthritis not responding to conventional treatments.

This study was conducted to assess the ability of the British Isles Lupus Assessment Group-2004 (BILAG-2004), the SLE Disease Activity Index-2K (SLEDAI-2K), the European Consensus Lupus Activity Measurement (ECLAM), and the Revised Systemic Lupus Activity Measure (SLAM-R) to detect the need to treatment change in daily clinical practice. One hundred and two patients with SLE were enrolled and followed up for 2 to 8 months and visited at least 3 times. Physician Global Assessment, BILAG-2004, SLEDAI-2K, SLAM-R, and ECLAM, were calculated in every visit. Treatment change, dependent variable, was categorized as decrease/no change vs. increase. The aforementioned indices, independent variables, were compared to learn their ability in predicting the treatment change. The probability of treatment change was measured by generalized linear-mixed effect model (GLMM) and generalized estimating equations (GEE). Adjusted odds ratios were calculated. Predictive power of indices was compared by area under the curve (AUC) in plots of sensitivity vs. 1-specificity and application of receiver operating characteristic curves (ROC). BILAG-2004 and SLEDAI-2K had substantial correlation with treatment change. Among different GLMM models, BILAG-2004 followed by SLEDAI-2K showed the highest associations with treatment change. Among various GEE models, similar findings were observed. Also, these 2 indices had the highest sensitivity (the largest AUC) towards treatment change; BILAG-2004 (AUC = 0.779, 95% CI = 0.710–0.848, p = 0.001) and SLEDAI-2K (AUC = 0.771, 95% CI = 0.698–0.843, p = 0.001). BILAG-2004 followed by SLEDAI-2K had the highest predictability of treatment change.

Background: Anti-chromatin antibodies have recently been described in patients with systemic lupus erythematosus (SLE) and it has been suggested that their presence is associated with lupus nephritis. Objective: To assess the prevalence and clinical associations of these antibodies in SLE. Methods: The presence of anti-chromatin antibodies in 100 patients with SLE was investigated by an enzyme linked immunosorbent assay (ELISA). To determine the specificity of these antibodies, 100 patients with primary Sjögren’s syndrome, 30 with primary antiphospholipid syndrome (APS), 10 with systemic sclerosis, and 100 normal controls were also tested. Results: Positive levels were detected in 69/100 (69%) patients with SLE. In contrast, they were found in only 8/100 (8%) of those with primary Sjögren’s syndrome, in 1/10 (10%) with systemic sclerosis, in 2/30 (7%) with primary APS, and in none of the 100 healthy controls. Patients with anti-chromatin antibodies had a twofold higher prevalence of lupus nephropathy than those without these antibodies (58% v 29%, p<0.01). A significant correlation was found between the levels of anti-chromatin antibodies and disease activity score as measured by the European Consensus Lupus Activity Measurement (ECLAM; p=0.011). Conclusions: The measurement of anti-chromatin antibodies appears to be a useful addition to the laboratory tests that can help in the diagnosis and treatment of SLE. These antibodies are both sensitive and specific for SLE, and are a useful marker for an increased risk of lupus nephritis.

Background: The survival rate in patients with systemic lupus erythematosus (SLE) has improved dramatically during the past four decades to 96.6% (five year) in the Erlangen cohort, but it is nearly three times as high as in an age and sex matched control population. Reasons for death are mainly cardiovascular diseases (37%) and infections (29%). Objective: To find risk factors existing at disease onset for a severe outcome in the Erlangen cohort. Patients and methods: By using a database of 338 patients with SLE from a single centre, documented at least one to 15 years and including Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage score data and index (SDI) and an activity score (European Consensus Lupus Activity Measurement (ECLAM)), a retrospective search was made for risk factors for a severe outcome like death, end stage renal disease (ESRD), and thromboembolic events (TE) in SLE. For this purpose, multivariable Cox regression models were analysed using the statistical package SPSS 10.0 for Windows. Results: The following were defined as risk factors for death at disease onset: male sex (p<0.001, relative risk (RR)=3.5), age >40 at disease onset (p<0.0001, RR=19.9), nephritis (p<0.05, RR=1.6), a reduction of creatinine clearance (p<0.001, RR=1.8), heart disease (p=0.05, RR=1.5), and central nervous system (CNS) disease (p=0.06, RR=1.6). An increase in the SDI of two or more points from the first to the third year of disease was the worst prognostic factor (p<0.0001, RR=7.7). The existence of Ro or nRNP antibodies, or both, was protective (p<0.05, RR =0.1). A low C3 (p<0.01 RR=3.0) and splenomegaly (p<0.01 RR=2.7) at disease onset turned out to be risk factors for ESRD besides a nephritis. In patients with hypertension (p<0.05) and/or high titres of dsDNA antibodies (>70 U/l) (p<0.01) and/or a mean ECLAM score of 4 (p<0.01) in the course of disease, a prevalence of ESRD was recorded in 9% (p<0.05) and 10% (p<0.01), and 8% (p<0.01) v 4% in the whole group. Analysis of risk factors at disease onset for TE identified positive lupus anticoagulant (p=0.17, RR=1.6), cryoglobulins (p<0.05, RR=1.8), and nephritis (p=0.05, RR=1.4), in addition to an age >40 at disease onset. Conclusions: A subgroup of patients in the Erlangen cohort with a typical clinical and serological phenotype at disease onset that is at high risk for a worse outcome was identified. Identification of these white patients at risk at disease onset will enable treatment to be intensified and thereby possibly prevent or better control late stage manifestations.

Systemic lupus erythematosus, SLE Disease Activity Index (SLEDAI), European Consensus Lupus Activity Measurement (ECLAM) INTRODUCTION: \"Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs and cells undergo damage initially mediated by tissue-binding autoantibodies and immune complexes\"1. The standardized measures used to access disease activity include SLE Disease Activity Index (SLEDAI), the European Consensus Lupus Activity Measurement (ECLAM), the British Isles Lupus Assessment Group (BILAG), the Lupus Activity Index (LAI) and the Systemic Lupus Activity Measure (SLAM) which are valid, reliable and comparable. The presence of traditional risk factors like sedentary lifestyle, obesity, hypercholesterolemia and nontraditional risk factors like renal impairment, high homo cysteine levels, chronic inflammation and low density lipid oxidation plays an important role in the management of the disease13, 14. Coronary artery disease risk factors in the Johns Hopkins Lupus Cohort: prevalence, recognition by patients, and preventive practices.

Background: With improved survival rates of patients with systemic lupus erythematosus (SLE), damage such as accelerated atherosclerosis gains increasing importance. Objective: To identify the prevalence of coronary artery calcifications (CAC) in asymptomatic patients. Methods: Electron beam tomography (EBT) was performed in 75 female patients with SLE aged <50. The results were correlated with traditional and SLE related factors associated with CAC. 49 women with symptomatic coronary heart disease (CHD) and 279 women without CHD were also analysed. Results: Overall, 21/75 (28%) patients had CAC. Low HDL cholesterol levels <1.40 mmol/l (p=0.03, OR=1.8, 67% v 39%) and cigarette smoking (p=0.01, OR=5.7, 76% v 44%) were identified as factors associated with CAC. Hypertension and high cholesterol were more common in women with CHD (p<0.01) than in those without CHD. SLE related factors were proteinuria (1331 v 465 mg/day, p=0.02), impaired renal function (p=0.02, OR=2.6, 26% v 6%), and high C3 levels (p=0.04, OR=1.8, 65% v 38%). High C3 levels were also more common in symptomatic CHD (p=0.02). The prevalence of Sm antibodies was lower in patients with CAC (15% v 42%, p=0.03). In a multivariate analysis, cigarette smoking, reduced renal function, high C3, and a cumulative steroid dose above 30 g were the most important CAC associated factors in the lupus cohort. Conclusion: A subgroup of patients with SLE with CAC without any clinical symptoms of CHD was identified by EBT. Therefore, EBT is useful for assessing asymptomatic atherosclerosis in this group.

Objectives: To study the role of different seasons in the disease activity of patients with systemic lupus erythematosus (SLE). Additionally, to evaluate whether the outdoor behaviour during the summer or a photoprovocation test affects disease activity. Methods: 33 patients with SLE were examined by a rheumatologist and a dermatologist at a university hospital in winter, spring, and summer. The activity of SLE was assessed by the ECLAM index. Their outdoor behaviour was recorded by a questionnaire during the summer. In the winter, 12 patients were photoprovoked by ultraviolet A and B radiation on a small skin area. Results: The ECLAM scores were higher in spring and tended to be higher in summer than in winter (p = 0.006 and p = 0.051). This finding, as well as the outdoor behaviour, were independent of the patients’ own impression of their photosensitivity. Overall, the sun protection actions were inadequate. The photoprovocation had no statistical effect on disease activity, but one patient had a violent exacerbation of SLE manifestations shortly after the photoprovocation. Conclusions: In the northern climate SLE may be activated during the sunny season. Therefore, more effort should be focused on sun protection of patients with SLE.

Objective: To evaluate a novel specific psychological intervention aimed at improving coping in patients with systemic lupus erythematosus (SLE). Methods: 34 community living SLE patients were recruited for the study. Intervention was undertaken in groups of up to eight patients and in two blocks over six months each. Eight patients were enrolled as a waiting list group. The 18 group sessions focused on information about the disease and specific problems of SLE patients, combining psychoeducative and psychotherapeutic elements. Psychological and medical evaluations were conducted at baseline and after three, six, and 12 months, using validated instruments. Results: The 34 SLE patients (91% female, mean age 42 years) improved significantly over a six month period on most of the psychological measuring instruments applied, such as depression, anxiety, and overall mental burden. The waiting list group showed no significant changes. Conclusions: Conceptualised psychoeducational support may produce a significant and sustained improvement in coping skills of SLE patients and hence in their quality of life.

Background: Receptors for IgG play an important part in immune complex clearance. Several studies have identified polymorphisms of receptors for the Fc fragment of IgG (FcγR) as genetic factors influencing susceptibility to disease or disease course of systemic lupus erythematosus (SLE). Objective: To examine these possibilities by evaluating a panel of clinical parameters in a cohort of 140 German patients with SLE for correlations with the FcγRIIa, IIIa, and IIIb polymorphisms in an explorative study. Methods: 140 German patients with SLE according to American College of Rheumatology (ACR) criteria and 187 German controls were genotyped for the FcγRIIa, IIIa, and IIIb polymorphisms. Associations between FcγR genotypes, combined genotypes and clinical as well as laboratory features were analysed. Results: No significant skewing of any of the three FcγR polymorphisms was seen in the German SLE cohort studied. Various clinical and serological parameters were found more frequently and at younger age in homozygous patients with the genotypes IIA-R/R131 or IIIA-F/F158 than in patients with IIA-H/H131 or IIIA-V/V158. These effects were even more pronounced in patients with the low binding combined phenotypes of the FcγRIIa, IIIa (double negative phenotypes) and FcγRIIa, IIIa, and IIIb (triple negative phenotypes). In patients with the double negative IIA and IIIA genotypes significantly higher frequencies of nephritis (63% v 33%) and proteinuria according to ACR criteria (58% v 11%), anaemia (84% v 55%), and anticardiolipin antibodies (63% v 22%) were found than in patients with the double positive genotypes. Patients with the IIA-R/R131 genotype and the double negative homozygous genotype had an earlier incidence of clinical symptoms, haematological and immunological abnormalities. Accordingly, SLE is diagnosed earlier in these patients, the difference reaching statistical significance only in the double negative v the double positive genotype (26.3 v 39.5 years) and the IIIA-F/F158 genotype v the rest (26.7 v 32.0 years). Most relevant is the fact that a higher median disease activity (ECLAM score) was demonstrated, both in the IIA-R/R131 homozygous (3.3 v 2.7) and the double negative (3.4 v 2.3) patients, reaching statistical significance in the first group. Conclusion: The results of this explorative study support the view that the FcγRIIa/IIIa and IIIb polymorphisms constitute factors influencing clinical manifestations and the disease course of SLE but do not represent genetic risk factors for the occurrence of SLE. Higher frequencies of clinical symptoms, haematological and immunological abnormalities as well as an earlier onset of clinical symptoms, haematological and immunological markers of active disease were found in patients with the IIA-R/R131 genotype and the double negative and triple negative genotypes.