Systemic Lupus Erythematosus Registry Research Articles

Improving Outcomes of Pediatric Lupus Care Delivery With Provider Goal-Setting Activities and Multidisciplinary Care Models.

The present study was undertaken to evaluate high-quality care delivery in the context of provider goal-setting activities and a multidisciplinary care model using an electronic health record (EHR)-enabled pediatric lupus registry. We then determined associations between care quality and prednisone use among youth with systemic lupus erythematosus (SLE). We implemented standardized EHR documentation tools to autopopulate a SLE registry. We compared pediatric Lupus Care Index (pLCI) performance (range 0.0-1.0; 1.0 representing perfect metric adherence) and timely follow-up 1) before versus during provider goal-setting activities and population management, and 2) in a multidisciplinary lupus nephritis versus rheumatology clinic. We estimated associations between pLCI and subsequent prednisone use adjusted for time, current medication, disease activity, clinical features, and social determinants of health. We analyzed 830 visits by 110 patients (median 7 visits per patient [interquartile range 4-10]) over 3.5 years. The provider-directed activity was associated with improved pLCI performance (adjusted β 0.05 [95% confidence interval (95% CI) 0.01, 0.09]; mean 0.74 versus 0.69). Patients with nephritis in multidisciplinary clinic had higher pLCI scores (adjusted β 0.06 [95% CI 0.02, 0.10]) and likelihood of timely follow-up than those in rheumatology (adjusted relative risk [RR] 1.27 [95% CI 1.02, 1.57]). A pLCI score of ≥0.50 was associated with 0.72-fold lower adjusted risk of subsequent prednisone use (95% CI 0.53, 0.93). Minoritized race, public insurance, and living in areas with greater social vulnerability were not associated with reduced care quality or follow-up, but public insurance was associated with higher risk of prednisone use. Greater attention to quality metrics is associated with better outcomes in childhood SLE. Multidisciplinary care models with population management may additionally facilitate equitable care delivery.

Medications impact different aspects of the quality of life of patients with systemic lupus erythematosus

AbstractPurposeIn this real‐world observational study, we analyzed the effects of different drugs on the quality of life (QoL) of patients with systemic lupus erythematosus (SLE).MethodWe identified patients in our prospective SLE Registry who received new medications. We measure QoL with MOS 36‐Item Short‐Form Health Survey (SF‐36), a generic health questionnaire, and SLEQOL, a disease‐specific instrument. We compared the patients' scores before and after initiation of treatment.ResultsWe identified 259 episodes of drug initiation in 193 SLE patients. SLEQOL registered statistically significant changes with intravenous cyclophosphamide (total score and the domains of physical functioning, activities, and self‐image), mycophenolate (total score, treatment, mood, and self‐image) and azathioprine (total score, activities, and mood), but not with cyclosporin A and hydroxychloroquine. Two SF‐36 subscales (general health and physical functioning) showed statistically significant improvement in the patients who received intravenous cyclophosphamide. Both instruments have floor effect. There was weak correlation between changes in the QoL instruments and the patient assessment of disease activity or an objective disease activity index.ConclusionSLEQOL distinguishes the differential effects on QoL of the various drugs. Treatment with intravenous cyclophosphamide and mycophenolate impacted the highest numbers of domains of SLEQOL, followed by azathioprine, cyclosporin A, and hydroxychloroquine. SLEQOL may be a useful outcome measure in SLE clinical trials.

Determinants of Cervical Cancer Screening Patterns Among Women With Systemic Lupus Erythematosus.

Women with systemic lupus erythematosus (SLE) are vulnerable to cervical dysplasia due to the persistence of human papillomavirus (HPV) infection. The objective of this cross-sectional retrospective study was to investigate the prevalence of cervical cancer screening according to the American Society for Colposcopy and Cervical Pathology (ASCCP) SLE-specific cervical cancer screening guidelines. We also aimed to identify SLE-specific determinants associated with ASCCP adherence. Women aged 21 to 64 years enrolled in our institutional SLE registry were included in the study. The electronic medical record was manually reviewed to determine whether the patient was up to date on screening and which organizational guideline was used, in addition to other clinical variables. Multivariable logistic regression was used to estimate adjusted odds ratios (ORs) for ASCCP-congruent screening for each baseline characteristic. This study included 118 women with SLE; 38% were up to date per ASCCP guidelines, 16% were up to date per non-ASCCP guidelines, and 46% were overdue for screening. Having a gynecologist and being actively treated with immunosuppressant therapies were both associated with an increased odds of being up to date per the ASCCP guidelines, while Hispanic ethnicity was associated with reduced odds. Only half of the women with SLE in our study had guideline-congruent cervical cancer screening. Current immunosuppression exposure, rather than SLE disease activity, was associated with an increased odds of being up to date according to ASCCP guidelines. This study suggests the need for increased awareness and consensus among interdisciplinary providers regarding SLE-specific cervical cancer screening.

Cardiovascular Manifestations, Imaging, and Outcomes in Systemic Lupus Erythematosus: An Eight-Year Single Center Experience in the United States.

Systemic lupus erythematosus (SLE) is a challenging autoimmune and multi-system condition. With advances in cardiovascular screening and therapies for SLE patients, we evaluated the cardiovascular characteristics, multi-modality imaging, and outcomes of SLE at our tertiary referral center over an 8year period. Consecutive patients from our SLE registry from April 2012 to March 2020 were retrospectively analyzed. Data pertaining to cardiovascular manifestations, investigations, management, and outcomes were assessed. We studied 258 SLE patients (mean age 42.2 ± 14.7 years); 233 (90.3%) were female. The main cardiac manifestations at index SLE clinic were pericardial disease in 33.3%, valve disease in 18%, cardiomyopathy in 9.6%, and stroke in 7.4%. During a mean follow-up of 3.0 ± 2.2years after index SLE clinic, there were 5 (1.9%) deaths, 24 (9.3%) cardiovascular events, and 44 (17.1%) SLE-related hospitalizations. A history of stroke and hypertension were independently associated with cardiovascular events, hazard ratio (HR) (95% confidence intervals (CI)) of 5.38 (1.41-20.6) and 3.31 (1.02-10.7), respectively, while younger age and lower albumin predicted SLE-related hospitalizations. Cardiovascular manifestations are prevalent in SLE, especially for pericardial, valvular, and atherosclerotic diseases. With contemporary SLE and cardiovascular management, subsequent adverse cardiovascular events were infrequent in this study.

POSC189 A Patient Registry-Based Cross-Sectional Study of the Burden and Management of Comorbid Psoriatic Arthritis (PsA) in Patients with Systemic Lupus Erythematosus (SLE)

Coexistence of PsA and SLE is considered rare. Given the potential for phototherapy or drug-induced exacerbation of SLE, or worsening psoriasis with some biologic treatments, this study sought to better understand the burden of real-world comorbidities critical to optimizing patient outcomes. The OM1 SLE Registry (OM1;Boston, MA) follows 46,900+ SLE patients in the U.S. managed by rheumatologists longitudinally w/deep clinical data, including laboratory, patient-reported and disease activity information, and linked claims, starting from 2013. Index date was set by the first encounter w/SLE diagnosis code and ≥18 months of baseline available. Comorbidities were defined by ≥2 outpatient diagnosis codes ≥30 days apart or one inpatient diagnosis code prior to index. Obesity was defined as BMI≥30 kg/m2. Medications were identified by prescriptions, administrations or fills. RAPID3 (Routine Assessment of Patient Index Data 3) at index was also explored. Study included 44,186 patients (92% female, mean age 51 years) from the OM1 SLE Registry, 703 (1.4%) of whom had documented PsA. Patients with co-morbid PsA were slightly older, with higher proportion male (12% vs. 8%), white (68% vs. 56%) and non-Hispanic (69% vs. 64%) patients. Patients with PsA were more likely to be obese (50% vs. 40%). Mean joint-related disease activity was moderate (mean RAPID-3=4) across patients. Proportion with other autoimmune conditions were similar. Recent treatment w/systemic steroids, methotrexate, leflunomide and TNFa and IL-17 or IL12/23 inhibitors was more common in SLE-PsA patients. Hydroxychloroquine was less commonly used in PsA patients (31% vs. 42%). Managing T-cell and B-cell-driven conditions simultaneously poses a clinical challenge, attempting to balance treating cutaneous and joint-related manifestations with exacerbating disease flares. Clinical development and availability of therapies with other mechanisms of action may provide important alternatives. Since clinical trials typically exclude many comorbid conditions, real-world data regarding these complex phenotypes provide important insights into tailored treatment strategies.

Utilization of glucocorticoids among White and Black patients with systemic lupus erythematosus: Observations from the enrollment visit of a prospective registry.

Black patients with systemic lupus erythematosus (SLE) face higher rates of morbidity and mortality compared to White patients. Long-term glucocorticoid use has been associated with worse health outcomes among patients with SLE. We sought to quantify chronic glucocorticoid use among Black and White patients with SLE within a prospective registry. Using enrollment data from a registry at a large academic institution, we compared glucocorticoid use among Black and White patients with SLE. Multivariable logistic regression of race and glucocorticoid use was performed, adjusting for covariates exhibiting a bivariate association with glucocorticoids at significance level p < 0.10. 114 White participants (mean age 45; standard deviation (SD) 15) and 59 Black participants (mean age 42; SD 14) were analyzed. White participants had mean SLEDAI-2K score of 3.7 (SD 5.2). Black participants had mean SLEDAI-2K scores of 6.3 (SD 6.0). Among Black participants, 43 (72%) utilized glucocorticoids compared to White participants 39 (34%) (unadjusted odds ratio (OR) 5.17; 95% confidence interval (CI) 2.59-10.33). We did not observe differences between unadjusted hydroxychloroquine (OR 0.69; 95% CI 0.28-1.65) or conventional disease-modifying anti-rheumatic drug (cDMARD) (OR 1.07; 95% CI 0.57-2.01) utilization among Black and White participants. SLEDAI-2K, disability, recent hospitalization, and past or present hydroxychloroquine or cDMARD use were included in a logistic regression model. Adjusting for covariates, Black participants were more likely to be on glucocorticoids (adjusted OR 5.69; 95% CI 2.17-14.96); p = 0.0004). Adjusting for disease activity and other medications, Black patients had more exposure to chronic glucocorticoids than White patients in the Cleveland Clinic SLE registry. These patients may face increased glucocorticoid-related morbidity, which could contribute significantly to long-term health outcomes and utilization of health care resources. Future research in larger, more diverse registries should be conducted to further characterize patterns of glucocorticoid use.

Prevalence of Systemic Lupus Erythematosus in the United States: Estimates From a Meta-Analysis of the Centers for Disease Control and Prevention National Lupus Registries.

Epidemiologic data on systemic lupus erythematosus (SLE) are limited, particularly for racial/ethnic subpopulations in the US. This meta-analysis leveraged data from the Centers for Disease Control and Prevention (CDC) National Lupus Registry network of population-based SLE registries to estimate the overall prevalence of SLE in the US. The CDC National Lupus Registry network includes 4 registries from unique states and a fifth registry from the Indian Health Service. All registries defined cases of SLE according to the American College of Rheumatology (ACR) 1997 revised classification criteria for SLE. Case findings spanned either 2002-2004 or 2007-2009. Given the heterogeneity across sites, a random-effects model was used to calculate the pooled prevalence of SLE. An estimate of the number of SLE cases in the US was generated by applying sex/race-stratified estimates to the 2018 US Census population. In total, 5,417 cases were identified as fulfilling the ACR SLE classification criteria. The pooled prevalence of SLE from the 4 state-specific registries was 72.8 per 100,000 person-years (95% confidence interval [95% CI] 65.3-81.0). The prevalence estimate was 9 times higher among females than among males (128.7 versus 14.6 per 100,000), and highest among Black females (230.9 per 100,000), followed by Hispanic females (120.7 per 100,000), White females (84.7 per 100,000), and Asian/Pacific Islander females (84.4 per 100,000). Among males, the prevalence of SLE was highest in Black males (26.7 per 100,000), followed by Hispanic males (18.0 per 100,000), Asian/Pacific Islander males (11.2 per 100,000), and White males (8.9 per 100,000). The American Indian/Alaska Native population had the highest race-specific SLE estimates, both among females (270.6 per 100,000) and among males (53.8 per 100,000). In 2018, an estimated 204,295 individuals (95% CI 160,902-261,725) in the US fulfilled the ACR classification criteria for SLE. A coordinated network of population-based SLE registries provides more accurate estimates of the prevalence of SLE and the numbers of individuals affected with SLE in the US in 2018.

Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) implies a more severe disease with more damage accrual and higher mortality.

Antiphospholipid antibodies (aPL) have been associated with organ damage and certain features in systemic lupus erythematosus(SLE) patients. Our aim was to investigate the differences between SLE patients according to the presence of aPL and/or clinical antiphospholipid syndrome (APS). Patients from the RELESSER-T registry were included. RELESSER-T is a Spanish multicenter, hospital-based, retrospective, SLE registry. We included 2398 SLE patients, 1372 of whom were positive for aPL. Overall 1026 patients were classified as SLE, 555 as SLE-APS and817 as SLE-aPL. Regarding cardiovascular risk factors, SLE-APS patients had higher rates of hypertension, dyslipidemia and diabetes than those with SLE-aPL and SLE (p < 0.001). SLE-APS patients showed higher rates of neuropsychiatric, cardiac, pulmonary, renal and ophthalmological manifestations than the other groups (p < 0.001). SLE-APS patients presented greater damage accrual with higher SLICC values (1.9 ± 2.2 in SLE-APS, 0.9 ± 1.4 in SLE-aPL and 1.1 ± 1.6 in SLE, p < 0.001) and more severe disease as defined by the Katz index (3 ± 1.8 in SLE-APS, 2.7 ± 1.7 in SLE-aPL and 2.6 ± 1.6 in SLE, p < 0.001). SLE-APS patients showed higher mortality rates (p < 0.001). SLE-APS patients exhibited more severe clinical profiles with higher frequencies of major organ involvement, greater damage accrual and higher mortality than SLE-aPL and SLE patients.

FRI0186 JOINT INVOLVEMENT AND DISEASE ACTIVITY IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS: CALCULATION OF SWOLLEN TO TENDER JOINT COUNT RATIO IN A REAL WORLD COHORT IN THE US

Background:Joint swelling and tenderness are common in patients with systemic lupus erythematosus (SLE). Swollen to tender joint count ratio (STR) is an index originally used in rheumatoid arthritis (RA) which assesses severity of disease activity based on 28 joint counts [1]. In RA, STR is a predictor of treatment response with a higher score indicating greater likelihood of responding.Objectives:To characterize SLE patients in a real-world cohort based on disease activity as defined by STR.Methods:The OM1 SLE Registry (OM1, Boston, MA) follows more than 37,000 SLE patients longitudinally with deep clinical data, including laboratory, patient-reported and disease activity information, and linked administrative claims, starting from 2013. Patients ≥16 years of age with swollen and tender joint counts based on 28 joints on the same encounter were included. STRs were calculated by inserting 1 if the denominator was 0 [2]. Patients were categorized by first available STR as having low (STR &lt;0.5), moderate (0.5 ≤ STR ≤ 1.0), and high (STR &gt;1.0) disease activity. Clinical characteristics were summarized by disease activity group. Definitions of SLE treatments were based on 2019 EULAR recommendations [3].Results:As of December 2019, there were 9,919 patients with at least one STR available in the OM1 SLE Registry. STR was low in 80.4%, moderate in 12.2%, and high in 7.4% of patients. Mean age overall was 52.1 years (standard deviation: 14.8), 92.1% were female, and 71.8% of 7,730 patients with known race were white. Clinical characteristics by STR group are described in Table 1. Antimalarial use decreased and immunosuppressant use increased with increasing STR. Use of select disease-modifying antirheumatic drugs (DMARDs) was higher among patients with moderate or high STR. Lupus nephritis was more common in patients with low STR. A higher proportion of patients with moderate STR had osteoarthritis. The proportion of patients with anxiety and depression decreased with increasing STR. On average, patient and physician global assessments from MDHAQ were higher for patients with moderate STR.Table 1.Clinical characteristics of patients with SLE by swollen:tender joint count ratio groupLowSTR &lt;0.5(N=7,970)Moderate0.5 ≤ STR ≤ 1.0(N=1,211)HighSTR &gt;1.0(N=738)Treatment prior to STR, n (%) Antimalarial5,106 (64.1%)702 (58.0%)427 (57.9%) Biologics (belimumab or rituximab)662 (8.3%)113 (9.3%)70 (9.5%) Immunosuppressants2,310 (29.0%)398 (32.9%)252 (34.1%) Select DMARDs635 (8.0%)165 (13.6%)94 (12.7%) Steroids4,437 (55.7%)785 (64.8%)434 (58.8%)Disease conditions prior to STR, n (%) Anxiety266 (3.3%)25 (2.1%)12 (1.6%) Depression1,127 (14.1%)149 (12.3%)80 (10.8%) Lupus nephritis984 (12.3%)117 (9.7%)72 (9.8%) Osteoarthritis2,336 (29.3%)393 (32.5%)193 (26.2%) Osteoporosis631 (7.9%)95 (7.8%)47 (6.4%)MDHAQ, N1,991388230MDHAQ, mean (SD) Patient global assessment (0-10)4.5 (2.9)5.3 (2.7)4.4 (2.8) Physician global assessment (0-10)2.8 (2.7)3.8 (2.6)2.8 (2.3)Conclusion:Differences in treatments received were apparent between patients of varying disease activity groups with trends towards increased use among patients with higher disease activity. Additional research is needed to determine the utility of this measure for assessing SLE-related outcomes.

PSY29 CHARACTERISTICS AND DISEASE MANAGEMENT IN A LARGE REAL WORLD LUPUS NEPHRITIS COHORT IN THE US

Lupus nephritis (LN) is a common but heterogenous serious manifestation of systemic lupus erythematosus (SLE) associated with increased morbidity and mortality. Some evidence suggests men with SLE more frequently develop LN and with greater severity. The heterogeneity of LN complicates development of new treatment strategies. The purpose of this study was to characterize LN patients compared to non-LN SLE patients in a large, longitudinal, diverse dataset. The OM1 SLE Registry (OM1, Boston, MA) follows more than 37,000 patients longitudinally with deep clinical data, including laboratory, patient-reported and disease activity information, starting from 2013. Registry patients were identified as having new onset or prevalent LN based on relevant diagnosis codes (e.g., glomerular disease in SLE, glomerulonephritis, chronic kidney disease, proteinuria; ≥ 2 codes at least 30 days apart) or documented dialysis or kidney transplant. As of November 2019, a total of 7267 (19.3%) of SLE patients met applied criteria for LN. Median age at SLE follow-up start was 51 years (IQR 38-63) with a median follow-up duration of 6 years. At least one eGFR value was available for 71% of patients and 82% had clinical notes available. A higher proportion of LN patients compared to non-LN patients were male (12.3% vs. 7.3%) and of black race (28.7% vs. 16.5%). Treatments included hydroxychloroquine (74.5%), mycophenolate mofetil (31.7%), azathioprine (14.3%) and cyclophosphamide (1.8%). Cardiovascular (CVD) risk factors were more common in LN patients such as hypertension (71.5% vs 37.1%), hyperlipidemia (41.9% vs. 25.4%), and type 2 diabetes (23.5% vs. 11.1%), and LN patients were more likely to have coronary artery disease (15.9% vs. 6.5%) and cerebrovascular disease (10.5% vs. 4.9%). Over 8% of LN patients had end stage renal disease. Understanding the heterogeneous characteristics and course of LN patients is critical to refining clinical trial populations and improving renal and CVD outcomes.

The current status of the problem with systemic lupus erythematosus in Kazakhstan

Objective: to analyze the provision of medical, including rheumatology, care in Kazakhstan, to study the incidence of systemic lupus erythematosus (SLE), and to develop a registry of patients with this condition.Material and methods. The investigators analyzed the guidelines for the organization of medical, including rheumatology, care in the republic and the official statistical materials of the Ministry of Health of the Republic of Kazakhstan in the period 2012 to 2017. Articles were searched to select activity indices, organ damages and to assess the quality of life and treatment programs in order to create a registry of patients with SLE.Results and discussion. The paper presents the basic principles of providing medical, including rheumatology, care in the republic. It gives data on the issues of providing SLE patients with medicines in outpatient and inpatient settings. It also analyzes trends in the incidence of SLE in the population of Kazakhstan in 2012 to 2017. There were 4,448 SLE patients, including 3,986 women; a comparative analysis of indicators demonstrated a 62.8% increase in the incidence of SLE from 2012 to 2017. The purpose and objectives of the registry of patients with SLE were substantiated.Conclusion. An analysis of morbidity rates suggests that SLE remains to be significant in the republic. The incidence of SLE has been noted to increase in the period from 2012 to 2017; there is a female preponderance (89.6%). The application of the SLE registry in clinical practice will be able to improve the diagnosis of the disease in the early stage and to prevent possible complications.

Malignancy in Pediatric-onset Systemic Lupus Erythematosus.

To determine cancer incidence in a large pediatric-onset systemic lupus erythematosus (SLE) population. Data were examined from 12 pediatric SLE registries in North America. Patients were linked to their regional cancer registries to detect cancers observed after cohort entry, defined as date first seen in the clinic. The expected number of malignancies was obtained by multiplying the person-years in the cohort (defined from cohort entry to end of followup) by the geographically matched age-, sex-, and calendar year-specific cancer rates. The standardized incidence ratio (SIR; ratio of cancers observed to expected) was generated, with 95% CI. A total of 1168 patients were identified from the registries. The mean age at cohort entry was 13 years (SD 3.3), and 83.7% of the subjects were female. The mean duration of followup was 7.6 years, resulting in a total observation period of 8839 years spanning the calendar period 1974-2009. During followup, fourteen invasive cancers occurred (1.6 cancers per 1000 person-yrs, SIR 4.13, 95% CI 2.26-6.93). Three of these were hematologic (all lymphomas), resulting in an SIR for hematologic cancers of 4.68 (95% CI 0.96-13.67). SIR were increased for both male and female patients, and across age groups. Although cancer remains a relatively rare outcome in pediatric-onset SLE, our data do suggest an increase in cancer for patients followed an average of 7.6 years. About one-fifth of the cancers were hematologic. Longer followup, and study of drug effects and disease activity, is warranted.

Effect of alcohol consumption and smoking on disease damage in systemic lupus erythematosus: data from the Korean Lupus Network (KORNET) registry.

Background We assessed correlations of smoking habits and alcohol consumption with disease activity or damage in patients with systemic lupus erythematosus (SLE). Methods A total of 505 patients with SLE were enrolled in the Korean Lupus Network (KORNET) SLE registry from January 2014 to January 2016. Disease activity and organ damage were measured by the SLE Disease Activity Index 2000 (SLEDAI-2K) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index, respectively. Multivariate logistic regression analysis was used to analyze associations with cutaneous lesions. Results There were no differences in SLEDAI-2K and SLICC/ACR damage indices according to either smoking status or alcohol consumption. More frequent cutaneous damage was observed in current alcohol drinkers compared with non-current alcohol drinkers ( p = 0.020). Cutaneous damage was significantly associated with alcohol consumption (odds ratio (OR) 4.048, 95% confidence interval (CI) 1.251-12.102, p = 0.020). Both low (1-5 glasses/week) and high (≥6 glasses/week) amounts of alcohol consumption had a significant impact on cutaneous damage compared with the absence of current alcohol consumption ( p = 0.033 and p = 0.027, respectively). Pairwise comparison of alcohol consumption and smoking status with cutaneous damage showed that only alcohol consumption was significantly associated with the presence of cutaneous damage, compared with non-current alcohol consumption and non-current smoking (OR 3.513, 95% CI 1.130-10.920, p =0.030). Conclusions Current alcohol consumption, but not smoking, might influence the development of cutaneous damage in patients with SLE.

Systemic lupus erythematosus in Spanish males: a study of the Spanish Rheumatology Society Lupus Registry (RELESSER) cohort.

Objective The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. Methods A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). Results Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women). Male/female ratio was 1/9. Age of onset of symptoms and age at diagnosis were higher in men than in women ( p < 0.001). Males were diagnosed earlier than females (p = 0.04) and had more cardiovascular comorbidities ( p < 0.001). Two hundred and thirty-six males (68%) with SLE required hospitalization in comparison with 1713 females (53%) ( p < 0.001). During follow-up, 208 patients died: 30 men (9.3%) and 178 women (5.9%) ( p = 0.02). As regards clinical manifestations, loss of weight ( p = 0.01), lymphadenopathies ( p = 0.02), and splenomegaly ( p = 0.02) were more common in male patients. Female patients were more likely to have inflammatory rash, alopecia, and arthritis ( p < 0.05). As for lung involvement, men with SLE had more pleural fibrosis ( p < 0.001) and pulmonary embolism ( p = 0.01). However, Raynaud's phenomenon was more common in women (35%) than in men (23.7%) ( p < 0.001); lupus nephritis was more common in men, being present in 155 (44.8%) of males versus 933 (29%) of females ( p < 0.001). Multivariate analysis showed that SLE patients with a high Charlson index (more than 3 points) and age > 50 years had a higher mortality (odds ratios 3.6 and 2.1, respectively). Furthermore, SLE patients who developed pulmonary hemorrhage, pulmonary hypertension, psychiatric involvement, complement deficiency, and hemophagocytic syndrome also had higher mortality, regardless of gender. Conclusion Patients with SLE over the age of 50 years have an increased risk of mortality. In Caucasians, age at diagnosis and symptom onset is higher in men than in women. The diagnostic delay is shorter in men. Male SLE patients present more cardiovascular comorbidities, and also more serositis, adenopathies, splenomegaly, renal involvement, convulsion, thrombosis, and lupus anticoagulant positivity than women.

Systemic Lupus Erythematosus Does Not Increase Risk of Adverse Events in the First 6 Months After Total Knee Arthroplasty.

Increasing numbers of patients with systemic lupus erythematosus (SLE) are undergoing total knee arthroplasty (TKA). Whether postsurgical adverse events (AEs) are higher in patients with SLE than patients with osteoarthritis (OA) is unknown. This study aims to compare AEs within 6 months of TKA. Patients in a single institution's arthroplasty and SLE registries who underwent TKA from 2007 to 2014 were eligible. SLE cases were matched 1:2 to OA on age, sex, year of TKA, and procedure type. AEs were collected through chart review and registry responses. Baseline characteristics were compared and regression analysis performed to determine predictors of AEs. Fifty-two SLE TKA were matched to 104 OA TKA. There was no difference in follow-up between groups. SLE patients had more comorbidities (≥1 Charlson-Deyo comorbidity: SLE 38.4% vs. OA 17.3%; P-value < 0.001) and steroid use (preoperative [SLE 28.8% vs. OA 1.9%, P-value < 0.001] and perioperative "stress-dose" [30.8% vs. 2.9%, P-value = 0.01]). SLE patients did not experience more major (SLE 25.0% vs. OA 19.2%; P-value = 0.41), minor (15.4% vs. 10.6%; P-value = 0.39), or total (38.5% vs. 27.9%; P-value = 0.18) AEs. AEs were not increased among patients on stress-dose steroids. In a multiple logistic regression analysis controlling for comorbidities and diagnosis, neither SLE (OR 1.61, 95% CI 0.74-3.50) nor >1 comorbidity (OR 1.05, 95% CI 0.46-2.39) was an independent risk factor for AEs. SLE is not an independent risk factor for increased AEs 6 months after TKA. Stress-dose steroid use does not heighten AE risk. These findings should inform recommendations for SLE patients considering TKA.

The contribution of antiphospholipid antibodies to organ damage in systemic lupus erythematosus.

The objective of this study was to assess the contribution of clinically significant antiphospholipid antibodies (aPL) to organ damage in systemic lupus erythematosus (SLE). Patients with disease duration of less than 10 years and at least 5 years of follow-up were identified from two SLE registries. A clinically significant antiphospholipid antibody (aPL) profile was defined as: positive lupus anticoagulant, anticardiolipin IgG/M ≥ 40 G phospholipid units (GPL)/M phospholipid units (MPL), and/or anti-β2-glycoprotein-I IgG/M ≥ 99th percentile on two or more occasions, at least 12 weeks apart. Organ damage was assessed by the Systemic Lupus International Collaborating Clinics Damage Index (SDI). Univariate and multivariate analysis compared SLE patients with and without SDI increase during a 15-year follow-up. Among 262 SLE patients, 33% had a clinically significant aPL profile, which was associated with an increased risk of organ damage accrual during a 5-year follow-up in univariate analysis, and during a 15-year follow-up in the multivariate analysis adjusting for age, gender, race, disease duration at registry entry, and time. In the multivariate analysis, older age at diagnosis and male gender were also associated with SDI increase at each time point. A clinically significant aPL profile is associated with an increased risk of organ damage accrual during a 15-year follow-up in SLE patients.

The African Lupus Genetics Network (ALUGEN) registry: standardized, prospective follow-up studies in African patients with systemic lupus erythematosus

The prevalence and severity of systemic lupus erythematosus (SLE) differs between ethnic groups and geographical regions. Although initially reported as rare, there is growing evidence that SLE is prevalent and runs a severe course in Africa. There is a paucity of prospective studies on African SLE patients. The African Lupus Genetics Network (ALUGEN) is a multicentred framework seeking to prospectively assess outcomes in SLE patients in Africa. Outcomes measured will be death, hospital admission, disease activity flares, and SLE-related damage. We will explore predictors for these outcomes including clinical, serological, socio-demographic, therapeutic and genetic factors. Further, we will investigate comorbidities and health-related quality of life amongst these patients. Data of patients recently (≤ 5 yrs) diagnosed with SLE will be collected at baseline and annual follow-up visits, and captured electronically. The ALUGEN project will facilitate standardized data capture for SLE cases in Africa, allowing participating centres to develop their own SLE registries, and enabling collaboration to enrich our understanding of inter-ethnic and regional variations in disease expression. Comprehensive, high-quality multi-ethnic data on African SLE patients will expand knowledge of the disease and inform clinical practice, in addition to augmenting research capacity and networking links and providing a platform for future biomarker and interventional studies.

SLE myopathy: a clinicopathological study.

To study the histological changes in muscle biopsy in patients diagnosed with systemic lupus erythematosus (SLE) and presenting with muscle symptoms. Patients with SLE presenting with muscle symptoms at presentation or during follow-up and treatment between January 2010 and May 2014 and who underwent muscle biopsy were included in the study. Demographic, clinical features, serological markers, and Electromyoneurography findings were collected. Muscle biopsies were evaluated for inflammation, fiber type abnormalities, vasculitis and interstitial changes according to 2004 European Neuromuscular Centre criteria. Fifteen patients underwent muscle biopsy in the study period. Clinical presentations included mucocutaneous (9), musculoskeletal (14), hematological (3), renal (8), hepatic (1) and central nervous system (CNS) (4) manifestations, and neuropathy (4), serositis (2), Raynaud's phenomenon (1) and infectious complications (8). Histological findings were perivascular/perimysial inflammation (7/15), type 1 predominance (7/15) and type 2 atrophy (13/15). Clinical evidence of myositis was seen in 8.8% of patients in the SLE registry, whereas histological evidence of myositis was seen in 46.66% of biopsied patients with muscle symptoms. There was no significant difference in clinical, serological, laboratory and muscle histology features between groups of patients with or without myositis. Type 2 atrophy was the predominant finding in SLE patients irrespective of time of muscle biopsy, treatment received and presence or absence of myositis. Histological evidence of myositis was found in 46.66% of SLE patients and there were no significant differences in clinical/laboratory features in patients with or without myositis. Type 2 atrophy was seen in patients irrespective of time of the biopsy, treatment received and presence or absence of myositis. Type 2 atrophy was considered the major cause for muscle symptoms.

Clinical and immunological pattern of systemic lupus erythematosus in men in a cohort of 2355 patients

To investigate the impact of gender on expression of systemic lupus erythematosus (SLE) in a cohort of 2355 SLE patients as one of the largest series of cases among the present reports. In this retrospective study we used medical records of all patients (239 male and 2116 female) of the SLE registry of Rheumatology Research Center (RRC), Tehran University of Medical science (TUMS), Iran. Both clinical and paraclinical manifestations of SLE patients have been registered in this database since 1976 and updated during their follow-up. Chi-square test was used to compare the clinical and paraclinical manifestations in men and women at disease onset and during the disease course. We used logistic regression to compute odds ratios with 95% confidence intervals. A P-value < 0.05 was considered as statistically significant. Mean age at disease onset was 25 ± 11.8 and 24.5 ± 10.3 years in men and women, respectively (P = 0.48). Comparison of clinical and immunological manifestations showed that male patients had a higher prevalence of mucocutaneous (43.5% vs. 33.7%, P = 0.005) and a lower prevalence of musculoskeletal symptoms (44% vs. 54.7%, P = 0.003) as the initial manifestation. During the disease course, discoid rash (25.9% vs. 13%, P = 0.000) and type IV lupus nephritis (23.4% vs. 18.1%, P = 0.03) were significantly more common, whereas arthritis (61.1% vs. 71.7%, P = 0.01) and leukopenia (28.5% vs. 35.8%, P = 0.024) were significantly less common in men. This study reveals gender influence on some manifestations of SLE. Considering sex differences is recommended in diagnostic and therapeutic features of the disease.

National Registry of Patients With Systemic Lupus Erythematosus of the Spanish Society of Rheumatology: Objectives and Methodology

ObjectiveTo describe the objectives, design and methods of the Spanish Society of Rheumatology systemic lupus erythematosus (SLE) registry (RELESSER). MethodsMulticenter, hospital-based registry, with retrospective collection of data from a large representative sample of adult patients with SLE (1997 ACR criteria) attending Spanish rheumatology services. The registry includes demographic data, frequent and infrequent (<1%) clinical manifestations, information about activity, damage, severity, comorbidity, treatments and mortality, collecting 359 variables per patient, with highly standardized definitions. We performed a preliminary descriptive analysis of the data. ResultsForty-five centers were involved and 4024 SLE patients (91% with ≥4 ACR criteria) have been included; 90% are women and 93% Caucasians, with a median age at diagnosis of 33 years, median disease duration: 120 months, median follow-up duration: 104 months; 3222 (81%) of the patients are in active follow-up and 591 (14%) were lost to follow-up. The median values of the SELENA-SLEDAI score, SLICC/ACR damage index and Katz severity index have been 2, 1 and 2, respectively. A total of 211 patients (6%) died. ConclusionsRELESSER represents the largest European SLE registry built to date, providing comprehensive and reliable information on SLE manifestations, disease status, comorbid conditions and treatments in daily clinical practice. RELESSER is constituted as a tool of great potential for multicenter clinical research in SLE.