Outcome Of Systemic Lupus Erythematosus Research Articles

Quality of care in systemic lupus erythematosus: the association between process and outcome measures in the Lupus Outcomes Study

ObjectivesAlthough process measures to assess quality of care in systemic lupus erythematosus (SLE) are available, their relationship to long-term outcomes has not been studied. Using a prospective, longitudinal cohort study,...

The impact of skin damage due to cutaneous lupus on quality of life

Patients with more severe cutaneous lupus erythematosus (CLE) have a poorer quality of life (QoL). Racial and ethnic disparities have been reported in disease activity and outcomes in systemic lupus erythematosus, but similar information is not available for CLE. To evaluate the impact of lupus-related skin damage on skin-specific QoL, and to analyse differences stratified by ethnic background. Data collected included sex, race, diagnosis and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and Skindex-29 scores. These parameters were analysed at the initial and last visits. CLASI damage scores (dyspigmentation and scarring) and activity scores were collected, grouped by ethnicity, and correlated with Skindex-29. Overall, 223 patients were analysed at baseline, with 141 completing more than one study visit. The majority of patients were white (63·7%), followed by African American (29·1%) and Asian American (4·0%). African American patients accounted for a disproportionate percentage of both localized (50%) and generalized discoid lupus erythematosus (DLE) (49%). Median CLASI damage scores differed significantly between the African American, white and Asian American patients, at both the first (8·5, 4·0, 7·0, respectively; P < 0·0001) and last visit (10·0, 6·0, 8·5, respectively; P < 0·01). CLASI damage scores in African Americans correlated with CLASI activity scores (Spearman r = 0·45, P = 0·0003). There was no significant correlation between CLASI damage scores and Skindex domains overall. Individually, dyspigmentation and scarring also did not have a significant effect on QoL. Ethnic differences in patients with CLE were found: African American patients exhibited a high rate of DLE and experienced damage early in their disease course, frequently in conjunction with disease activity.

Prolonged improvement of childhood onset systemic lupus erythematosus following systematic administration of rituximab and cyclophosphamide

BackgroundAlthough the combination of cyclophosphamide and rituximab has been utilized in case reports, there are no previous reports of the long term outcome of SLE treated systematically with this regimen. We report a pilot study to evaluate the efficacy of a systematically administered course of rituximab and cyclophosphamide over an eighteen month period to provide sustained improvement in childhood onset systemic lupus erythematosus (SLE).FindingsTwelve patients with childhood onset lupus nephritis or corticosteroid resistant SLE received systematic treatment with a combination of rituximab (750 mg/M2 up to 1 gram) and cyclophosphamide (750 mg/M2: no patient exceeded 1.8 M2). Two administrations of rituximab and cyclophosphamide, two weeks apart, were administered at the start of study, six months later, and eighteen months later. Clinical data were collected and analyzed after sixty months of follow up. There was sustained improvement in all clinical parameters with a dramatic reduction in both mean SLEDAI score (10.1 to 1 at one year and 0 at five years p<0.005) and mean daily prednisone dosage (29.7 mg/day to 12.7 by one year and 7.0 mg/day at five years p<0.005), with sustained improvement in mean C3 (55.5 mg/ml to 113 at one year and 107.5 at five years p<0.001) which was maintained through sixty months of follow up. Serum immunoglobulin levels were transiently depressed but mean values were within the normal range for both IgG and IgM at one and five years. Few complications were observed (two episodes of febrile neutropenia during the first year of treatment were the only serious adverse events) and patients routinely reported sustained wellbeing.ConclusionsThis pilot study demonstrates that a systematically administered course of rituximab and cyclophosphamide over an eighteen month period provided sustained relief for patients with childhood onset SLE which was maintained over a sixty month period, while minimizing the need for corticosteroids, without excessive toxicity.

Specific features of systemic lupus erythematosus in males: case reports and literature data

The paper presents the data available in the literature on the specific features of systemic lupus erythematosus (SLE) in male patients, analyzes the clinical picture and outcomes of SLE in the men long followed up at the Research Institute of Rheumatology, Russian Academy of Medical Sciences, and gives clinical examples.

Pulmonary Hypertension in Systemic Lupus Erythematosus

Pulmonary hypertension (PH) is a life-threatening complication of systemic lupus erythematosus (SLE). Pulmonary hypertension in SLE has a variety of causes. Diagnosing early and defining the cause of PH accurately can provide better clinical outcome in SLE. We investigated the causes and characteristics of PH in patients with SLE. One hundred twenty-one patients with SLE who had a visit in a 6-month period were assessed retrospectively. Patients who ever had a systolic pulmonary arterial pressure of 40 mm Hg or greater by Doppler echocardiography were considered to have PH. Among 122 patients, 65 had echocardiography for some reason, and 10 (8.2%) were diagnosed as having PH by echocardiographic examination. This number reduced to 9 (7.4%) when we excluded the patient with normal pulmonary artery pressure at right heart catheterization. Causes of PH were as follows: thromboembolic events in 4 patients (44.4%) (2 of them had chronic thromboembolic PH), left-sided heart disease in 2 patients (22.2%), pulmonary arterial hypertension in 1 patient (11.1%), high cardiac output state in 1 patient (11.1%), and transient elevation of systolic pulmonary artery pressure in 1 patient (11.1%) who had a history of venous thromboembolism. Venous thromboembolic disease was significantly higher in patients with SLE with PH in comparison to patients with SLE without PH (7 patients [6.3%] vs 5 patients [50.0%]; P = 0.001). All patients improved clinically during their short-term follow-up. Patients with SLE are at increased risk for PH. This study highlights the complexity of the differential diagnosis of PH in patients with SLE once again and emphasizes the importance of pulmonary thromboembolism as a cause of PH. One should investigate patients with SLE with unexplained symptoms and/or signs related to PH for possible treatable causes.

Ethnicity in systemic lupus erythematosus (SLE): its influence on susceptibility and outcomes

Ethnicity is a biological and a social construct which encompasses ancestral genes, cultural, geographic and socioeconomic characteristics shared within a population. It is clear that no homogeneous racial groups exist within the human race as demonstrated when examining ancestry informative markers. Both the genetic and non-genetic components of ethnicity exert influence in the expression and outcome of systemic lupus erythematosus (SLE), including disease activity, damage accrual, work disability and mortality. Although it is difficult to determine the extent to which the differences observed in these parameters are caused by genetic or non-genetic factors, early in the disease genetic factors seem to play a more important role as determinants of the differences observed between SLE patients from various ethnic groups. Over the course of the disease, non-genetic factors seem to play a more important role. By and large, SLE is more frequent and more severe with higher disease activity and more damage accrual in non-Caucasian populations (Hispanics, African descendants and Asians) than in Caucasians. To overcome these differences it is necessary to optimize health care access to disadvantaged populations and use innovative tools to increase disease awareness and improve treatment adherence.

TULUP (TUnisian LUPus): a multicentric study of systemic lupus erythematosus in Tunisia

Clinical features of systemic lupus erythematosus (SLE) have been described from different geographical regions around the world. However, data from North African countries, including Tunisia, are scarce. The aim of this retrospective multicenter study was to analyze demographic, clinical, laboratory features and outcome of SLE in Tunisia throughout 14 Departments of Internal Medicine and to compare them with those of other ethnic and geographic groups. Seven hundred and forty-nine cases of SLE were recorded (American College of Rheumatology criteria) during a 17-year period (1989-2006). They were 676 women and 73 men with an average age at SLE onset of approximately 30.66 years. Our Tunisian patients were characterized by a high frequency of photosensitivity (67.6%), malar rash (68.7%), renal involvement (49.5%) and anti-Sm antibodies (44.8%). Infections were the main complications. Fifty-six (7.5%) patients died during the study period. Potential limitations and biases in our study need discussion. Specific recruitment of patients in tertiary referral centers may be the source of selection bias and adding to the frequency of moderate or even severe diseases. The therapeutic management and outcome monitoring were heterogeneous due to the fact that patients were evaluated by different doctors. However, this study remains the most representative of Tunisian SLE patients recruited from all parts of Tunisia.

The outcome of patients with renal involvement in pediatric-onset systemic lupus erythematosus – a 20-year experience in Asia

Systemic lupus erythematosus (SLE) predominantly affects women of childbearing age, but 15-20% of cases are diagnosed during childhood. It is important for physicians to understand the epidemiology and clinical presentation for early detection and diagnosis of this disease in difference races. The aim of this retrospective review was to provide a 20-year experience for initial clinical and laboratory manifestations and outcomes in pediatric-onset SLE (pSLE) in a medical center in Asia. We reviewed medical records between April 1990 and June 2012 of patients with a diagnosis of International Classification of Diseases, Ninth Revision (ICD-9) code 710.0 (SLE), who admitted or received follow-up in the Department of Pediatrics at Chang Chung Memorial Hospital. Patients with a diagnosis of SLE prior to their 18th birthday and followed up at our hospital were eligible for inclusion in this study. Medical records regarding age, gender, date of birth and diagnosis, clinical manifestations at diagnosis, laboratory results, image studies and the classification criteria were reviewed. Patients received regular outpatient department follow-up and laboratory survey every 1-6 months. The study cohort consisted of 189 patients; 164 females (86.87%) and 25 males (13.23%). The overall mean age at pSLE diagnosis was 12.62 ± 2.77 years. The most common clinical symptom was malar rash, followed by arthritis and oral ulcers. There was no significant difference in clinical and laboratory manifestations between females and males. More than half of the patients presented with renal involvement initially. The most common histological finding was Class IV lupus nephritis (LN), especially in males (p = 0.034) and young age. Even with severe LN, the rate of end-stage renal disease (ESRD) was low if adequate treatment was initiated. The 5, 10 and 15-year ESRD-free survival rates were 95.4%, 94.0% and 89.9% in patients with biopsy-proven LN. However, infection was the leading cause of mortality. Therefore, aggressive treatment for major organ involvement is important, but physicians must also be aware of fatal infection. The overall survival rates were 5 years: 93.4% and 10-20 years: 89.6%.

Physician-patient interactions and outcomes in systemic lupus erythematosus (SLE): a conceptual model

The objective was to develop a conceptual model illustrating the relationships between the physician-patient relationship and patient outcomes, including health status and regimen satisfaction, in systemic lupus erythematosus (SLE). This was a cross-sectional survey of a geographically diverse sample of adults with SLE in the United States. Patients completed a Web-based survey that focused on physician interactions, clinical management, and patient outcomes, including patient perception of treatment regimen and health status. All survey variables related to physician interactions and patient perceptions of their health and satisfaction were evaluated for incorporation into a patient-centered model using cluster analysis. Structural equation modeling (SEM) was conducted to assess the inter-relationships observed among the variables to inform the development of a conceptual model of SLE patient-centered care. A total of 302 SLE patients completed the survey. The majority of patients were female (94.3%) with a mean age of 46 years. The cluster analysis resulted in six main factors: 1) physician interactions, 2) current health and hope, 3) satisfaction with treatment, 4) bedside manner, 5) discussion of lupus impacts during physician visits, and 6) steroid treatment. The significant relationships among the factors showed that positive physician interactions, such as including the patient in treatment decisions, were associated with higher satisfaction with treatment regimen and patients feeling that SLE was well controlled, a more favorable perception of current health, and being more hopeful about future health. Among the components of physician interactions, setting goals with patients is particularly important, as this was significantly associated with the patient being more hopeful about future health. Being steroid free was significantly related to higher treatment satisfaction. The study findings informed a conceptual model of SLE patient-centered care that may be used to create more targeted education programs in the management of SLE, with the goal to improve patient outcomes.

Validation and evaluation of the German Brief Index of Lupus Damage (BILD) – a self-reported instrument to record damage in systemic lupus erythematosus

Damage is a very important predictor for outcome in systemic lupus erythematosus (SLE) and should be routinely documented. Patient-reported assessments for damage are rare and neither the Lupus Damage Index Questionnaire (LDIQ) nor the Brief Index of Lupus Damage (BILD) is validated in German language. Our aim was to validate the BILD in German language and evaluate its use as a patient-administered instrument. We translated and adapted the BILD questionnaire to use it as a self-administered questionnaire for German-speaking SLE patients. It was applied to SLE outpatients at an academic centre and compared to the SLICC/SDI and other lupus outcome parameters. The German BILD showed as strong a correlation with the SLICC/SDI as the original version of the BILD and a superior correlation compared to the LDIQ. It scored significantly higher with an increase of age, disease duration or disease activity, with a lower functional status or overall health and a higher probability of receiving an incapacity pension. The German version of the BILD shows a comparable validity to the original BILD with even higher correlation to physician-reported damage even when used as a self-administered questionnaire. Hence it represents a promising instrument to survey damage in clinical routine as well as in clinical and epidemiological studies.

AB0674 Higher frequency and worse outcome of lupus nephritis in korean male

BackgroundFemale preponderance is a well-known characteristic of systemic lupus erythematosus (SLE). Males with SLE are a minority group but distinguishable characteristics have been reported. Gender may influence the clinical features...

Fatigue, health-related quality of life and other patient-reported outcomes in systemic lupus erythematosus

This chapter aims to evaluate current knowledge of the burden of systemic lupus erythematosus (SLE) on individual patients, with a particular focus on health-related quality of life (HRQoL), activities of daily living (ADLs), individual symptoms, such as fatigue and pain, work disability and employment. A literature search was performed in Medline (PubMed) and a qualitative analysis was done of all publications relating to the burden of SLE (January 2000 to May 2010, updated in June 2013) matching the following inclusion criteria: prospective studies involving ≥100 patients with SLE; studies focussing on QoL, ADL, function/disability, patient perceptions/experience of their illness, physical/psychological/social impact, unmet needs, work disability/employment status or specific signs/symptoms expected to impact on HRQoL/ADL; studies predominantly involving patients of Caucasian ethnicity; and studies based in Europe, North America or Australia. Studies in juvenile patients; studies of the impact of obesity; studies of non-pharmacological interventions, dietary supplements or alternative medicines; and health-economic analyses were excluded. The present literature analysis showed that SLE has a considerable impact on the HRQoL of patients and their ability to carry out normal daily activities, resulting in a high prevalence of disability. The HRQoL of patients with SLE is consistently lower than that of matched healthy control subjects or patients with other chronic diseases. Predictors of poor HRQoL in patients with SLE include older age, fatigue and the presence of co-morbid neurological or psychiatric disorders, particularly depression or anxiety. Conversely, clinical measures of disease activity and organ damage are poor indicators of patients' HRQoL. Even though current evidence gives an insight into the HRQoL in patients with SLE, there are still many areas where results need to be confirmed or clarified and therefore there is the need to conduct further studies including trials with HRQoL as a primary objective.

SP0139 Predictors for Outcome of Juvenile SLE

Approximately 20% of patients withy systemic lupus erythematosus (SLE) have disease which begins in childhood. Several series have reported that childhood-onset SLE (cSLE) is often more severe than adult disease...

Clinical presentations and outcomes of systemic lupus erythematosus patients with infection admitted to the intensive care unit

The objective of this article is to investigate clinical presentations and outcomes of systemic lupus erythematosus (SLE) patients with infection admitted to the intensive care unit (ICU). SLE patients with infection, SLE patients with noninfectious causes, and non-SLE patients with infection were identified from the Cooper University Hospital Project IMPACT database between 2002 and 2010. We examined demographic data, APACHE II scores, physiologic data, laboratory data, length of stay in the ICU and hospital, and mortality of the three groups. Twenty-five SLE patients with infection, 45 SLE patients with noninfectious causes, and 1466 non-SLE patients with infection were included in the study. SLE patients with infection had higher APACHE II scores, higher maximum temperature, higher minimum and maximum heart rate (HR), lower minimum and maximum systolic blood pressure (SBP), and longer ICU length of stay in comparison to SLE patients with noninfectious causes. There were no statistical differences in white blood cell (WBC) count. SLE patients with infection had a higher mortality compared to SLE patients with noninfectious causes. There was no difference in mortality between SLE patients with infection and non-SLE patients with infection. SLE patients with infection in the ICU had a higher mortality and a higher APACHE II score compared to SLE patients with noninfectious causes in the ICU. Their physiologic signs including temperature, HR, and SBP were more reflective of infection than their WBC count.

Interleukin-17 Expression Positively Correlates with Disease Severity of Lupus Nephritis by Increasing Anti-Double-Stranded DNA Antibody Production in a Lupus Model Induced by Activated Lymphocyte Derived DNA

Lupus nephritis is one of the most serious manifestations and one of the strongest predictors of a poor outcome in systemic lupus erythematosus (SLE). Recent evidence implicated a potential role of interlukin-17 (IL-17) in the pathogenesis of lupus nephritis. However, the correlation between IL-17 expression level and the severity of lupus nephritis still remains incompletely understood. In this study, we found that serum IL-17 expression level was associated with the severity of lupus nephritis, which was evaluated by histopathology of kidney sections and urine protein. Of note, we showed that enforced expression of IL-17 using adenovirus construct that expresses IL-17 could enhance the severity of lupus nephritis, while blockade of IL-17 using neutralizing antibody resulted in decreased severity of lupus nephritis. Consistently, we observed an impaired induction of lupus nephritis in IL-17-deficient mice. Further, we revealed that IL-17 expression level was associated with immune complex deposition and complement activation in kidney. Of interest, we found that IL-17 was crucial for increasing anti-double-stranded DNA (dsDNA) antibody production in SLE. Our results suggested that IL-17 expression level positively correlated with the severity of lupus nephritis, at least in part, because of its contribution to anti-dsDNA antibody production. These findings provided a novel mechanism for how IL-17 expression level correlated with disease pathogenesis and suggested that management of IL-17 expression level was a potential and promising approach for treatment of lupus nephritis.

Systemic lupus erythematosus in Saudi children: long-term outcomes

To report the long-term outcome of Saudi children with systemic lupus erythematosus (SLE). Charts of all children with SLE treated between 1990 and 2010 at King Faisal Specialist Hospital and Research Center Riyadh, were reviewed. The long-term outcome measured by pediatric adaptation of the Systemic Lupus International Collaborating Clinics American College of Rheumatology Damage Index (pSDI) and death related to SLE were determined. The data included: gender, age at disease onset, clinical features and treatment at last follow-up visit. One hundred and fifty-two patients (129 girls and 23 boys) were included. The mean age at onset of SLE was 8.8±2.6years, while the mean age at diagnosis was 9.5±2.6years and the mean disease duration was 7.5±4.6years. All patients were treated with corticosteroid and immunosuppressive drugs. Eighty (52.6%) patients had damage with a mean SDI score of 1.3±1.7. Damage accrual was mostly in the growth (26.8%), renal (17.1%) and neuropsychiatric (15.8%) domains. Due to progressive renal disease, 14 patients required dialysis; five of them underwent renal transplant. There were nine deaths related to SLE, eight of them due to infection. Based on logistic regression, patient disease damage was significantly associated with young age at disease onset and long disease duration. Similarly, death related to SLE was influenced by early-onset disease. In contrast, gender, disease duration and therapy did not affect the suggested outcome measures. Our results are comparable to reports from other tertiary centers. Early-onset disease probably influences the long-term outcome of SLE in children. Infection remains an important cause of death in children with SLE.

PHS61 Patient Involvement in Treatment Decisions and Its Perceived Impact on Patient Outcomes in Systemic Lupus Erythematosus (SLE): A Comparison of Physician and Patient Perspectives

PHS61 Patient Involvement in Treatment Decisions and Its Perceived Impact on Patient Outcomes in Systemic Lupus Erythematosus (SLE): A Comparison of Physician and Patient Perspectives

Analysis of systemic lupus erythematosus sub-phenotype data for genetic association

The purpose of this review is to summarise the current understanding of genetic associations with systemic lupus erythematosus (SLE) sub-phenotypes, and to discuss how improvement in statistical methodology and data quality will increase our understanding of SLE generally. Recent studies of genetic association with SLE sub-phenotypes have found evidence of heterogeneity of effect over phenotype. These results suggest that the associations observed in many studies for the more general SLE outcome are a simplification of a complex multivariate genetic association.There has been some recent development in statistical methodology for the analysis of association across related phenotypes. These methodologies should be utilised more by the scientific community investigating SLE susceptibility loci as they are more powerful than simple case-control designs at detecting genetic effects that are heterogeneous across phenotypes. A focus on sub-phenotype data will lead to a better understanding of the biology of disease as we can investigate what aspects of the disease are affected by particular genes. This will give us the opportunity to learn how genetics can contribute to better diagnosis and prognosis of complex disease.

The impact of rural residency on the expression and outcome of systemic lupus erythematosus: data from a multiethnic Latin American cohort

The objective of this paper is to examine the role of place of residency in the expression and outcomes of systemic lupus erythematosus (SLE) in a multi-ethnic Latin American cohort. SLE patients (< two years of diagnosis) from 34 centers constitute this cohort. Residency was dichotomized into rural and urban, cut-off: 10,000 inhabitants. Socio-demographic, clinical/laboratory and mortality rates were compared between them using descriptive tests. The influence of place of residency on disease activity at diagnosis and renal disease was examined by multivariable regression analyses. Of 1426 patients, 122 (8.6%) were rural residents. Their median ages (onset, diagnosis) were 23.5 and 25.5 years; 85 (69.7%) patients were Mestizos, 28 (22.9%) Caucasians and 9 (7.4%) were African-Latin Americans. Rural residents were more frequently younger at diagnosis, Mestizo and uninsured; they also had fewer years of education and lower socioeconomic status, exhibited hypertension and renal disease more frequently, and had higher levels of disease activity at diagnosis; they used methotrexate, cyclophosphamide pulses and hemodialysis more frequently than urban patients. Disease activity over time, renal damage, overall damage and the proportion of deceased patients were comparable in rural and urban patients. In multivariable analyses, rural residency was associated with high levels of disease activity at diagnosis (OR 1.65, 95% CI 1.06-2.57) and renal disease occurrence (OR 1.77, 95% CI 1.00-3.11). Rural residency associates with Mestizo ethnicity, lower socioeconomic status and renal disease occurrence. It also plays a role in disease activity at diagnosis and kidney involvement but not on the other end-points examined.

Cardiovascular disease and cognitive dysfunction in systemic lupus erythematosus.

Cognitive dysfunction and cardiovascular disease are common and debilitating manifestations of systemic lupus erythematosus (SLE). In this study, we evaluated the relationship between cardiovascular events, traditional cardiovascular risk factors, and SLE-specific risk factors as predictors of cognitive dysfunction in a large cohort of participants with SLE. Subjects included 694 participants from the Lupus Outcomes Study (LOS), a longitudinal study of SLE outcomes based on an annual telephone survey querying demographic and clinical variables. The Hopkins Verbal Learning Test-Revised and the Controlled Oral Word Association Test were administered to assess cognitive function. Multiple logistic regression was used to identify cardiovascular events (myocardial infarction, stroke), traditional cardiovascular risk factors (hypertension, hyperlipidemia, diabetes mellitus, obesity, smoking), and SLE-specific risk factors (antiphospholipid antibodies [aPL], disease activity, disease duration) associated with cognitive impairment in year 7 of the LOS. The prevalence of cognitive impairment as measured by verbal memory and verbal fluency metrics was 15%. In adjusted multiple logistic regression analyses, aPL (odds ratio [OR] 2.10, 95% confidence interval [95% CI] 1.3-3.41), hypertension (OR 2.06, 95% CI 1.19-3.56), and a history of stroke (OR 2.27, 95% CI 1.16-4.43) were significantly associated with cognitive dysfunction. In additional analyses evaluating the association between these predictors and severity of cognitive impairment, stroke was significantly more prevalent in participants with severe impairment when compared to those with mild or moderate impairment (P = 0.036). These results suggest that the presence of aPL, hypertension, and stroke are key variables associated with cognitive impairment, which may aid in identification of patients at greatest risk.