Objective. To compare differences in long-term outcome between adults with childhood-onset (age at diagnosis <18 years) systemic lupus erythematosus (SLE) and with adult-onset SLE. Using self-report data, differences in organ involvement and disease morbidity, current disease status and activity, past and current medication use, and number of physician visits were compared, based on age at diagnosis of SLE. Results. Average disease duration for the cSLE and aSLE subgroups was 16.5 and 13.4 years, respectively, and mean age at followup was 30.5 and 49.9 years, respectively. When compared with aSLE subjects, cSLE subjects had a higher frequency of SLE-related renal disease, whereas aSLE subjects were more likely to report a history of pulmonary disease. Rates of clotting disorders, seizures, and myocardial infarction were similar between the 2 groups. At followup, cSLE subjects had lower overall disease activity, but were more likely to be taking steroids and other immunosuppressive therapies. The total number of yearly physician visits was similar between the 2 groups, although cSLE subjects had a higher number of nephrology visits. Conclusion. This study demonstrates important differences in the outcomes of patients with cSLE and aSLE, and provides important prognostic information about long-term SLE disease activity and treatment.

Methods. Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients.Results. MD diagnoses of 117 patients (82 of whom were female) were WG (n ‫؍‬ 76), microscopic polyangiitis (n ‫؍‬ 17), ANCA-positive pauci-immune glomerulonephritis (n ‫؍‬ 5), Churg-Strauss syndrome (n ‫؍‬ 2), and unclassified vasculitis (n ‫؍‬ 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were

Objective To formulate consensus treatment plans (CTPs) for induction therapy of newly-diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (jSLE). Methods A structured consensus formation process was employed by the members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) after considering the existing medical evidence and current treatment approaches. Results After an initial Delphi survey (response rate 70%), a 2-day consensus conference, and two follow-up Delphi surveys (response rates 63–79%), consensus was achieved for a limited set of CTPs addressing the induction therapy of proliferative LN. These CTPs were developed for prototypic patients defined by eligibility characteristics, and included immunosuppressive therapy with either mycophenolic acid orally twice per day, or intravenous cyclophosphamide once per month at standardized doses for six months. Additionally, the CTPs describe three options for standardized use of glucocorticoids; including a primarily oral, a mixed oral/intravenous, and a primarily intravenous regimen. There was consensus on measures of effectiveness and safety of the CTPs. The CTPs were well accepted by the pediatric rheumatology providers treating children with LN, and up to 300 children per year in North America are expected to be candidates for the treatment with the CTPs. Conclusion CTPs for induction therapy of proliferative LN in jSLE based on the available scientific evidence and pediatric rheumatology group experience have been developed. Consistent use of the CTPs may improve the prognosis of proliferative LN, and support the conduct of comparative effectiveness studies aimed at optimizing therapeutic strategies for proliferative LN in jSLE.

Objective To examine childhood-onset disease as a predictor of mortality in a cohort of adult patients with systemic lupus erythematosus (SLE). Methods Data were derived from the University of California Lupus Outcomes Study, a longitudinal cohort of 957 adult subjects with SLE that includes 98 subjects with childhood-onset SLE. Baseline and follow-up data were obtained via telephone interviews conducted between 2002-2007. The number of deaths during 5 years of follow-up was determined and standardized mortality ratios (SMRs) for the cohort, and across age groups, were calculated. Kaplan-Meier life table analysis was used to compare mortality rates between childhood (defined as SLE diagnosis <18 years) and adult-onset SLE. Multivariate Cox proportional hazard models were used to determine predictors of mortality. Results During the median follow-up period of 48 months, 72 deaths (7.5% of subjects) occurred, including 9 (12.5%) among those with childhood-onset SLE. The overall SMR was 2.5 (CI 2.0-3.2). In Kaplan-Meier survival analysis, after adjusting for age, childhood-onset subjects were at increased risk for mortality throughout the follow-up period (p<0.0001). In a multivariate model adjusting for age, disease duration and other covariates, childhood-onset SLE was independently associated with an increased mortality risk (hazard ratio [HR]: 3.1; 95% confidence interval [CI]: 1.3-7.3), as was low socioeconomic status measured by education (HR: 1.9; 95% CI 1.1-3.2) and end stage renal disease (HR: 2.1; 95% CI 1.1-4.0). Conclusion Childhood-onset SLE was a strong predictor of mortality in this cohort. Interventions are needed to prevent early mortality in this population.

Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding.

Objective. To estimate health care costs and costs associated with changes in work productivity among persons with systemic lupus erythematosus (SLE) in the US. Methods. Data were derived from the University of California, San Francisco Lupus Outcomes Study. Participants provided information on their health care resource use and employment. Cost estimates were derived for both direct health care costs and costs related to changes in work productivity. Direct health care costs included costs for hospitalizations, emergency department services, physician visits, outpatient surgical procedures, dialysis, and medications. Productivity costs were estimated by measuring changes in hours of work productivity since diagnosis of SLE; these estimates were also compared with normal US population data. Results. For the total population of participants, the mean annual direct cost was $12,643 (2004 US dollars). The mean annual productivity cost for subjects of employment age (>18 and <65 years) was $8,659. The mean annual total cost (direct and productivity) for subjects of employment age was $20,924. Regression results showed that greater disease activity, longer disease duration, and worse physical and mental health were significant predictors of higher direct costs; older age predicted lower direct costs. Older age, greater disease activity, and worse physical and mental health status were significant predictors of higher costs due to changes in work productivity. Conclusion. Both direct health care costs and costs associated with changes in work productivity are substantial and both represent important contributors to the total costs associated with SLE.

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthropathy of childhood. Juvenile idiopathic arthritis is believed to be a complex genetic trait influenced by both genetic and environmental factors. Twin and family studies suggest a substantial role for genetic factors in the predisposition to JIA. Describing the genetics is complicated by the heterogeneity of JIA; the International League of Associations for Rheumatology (ILAR) has defined seven categories of JIA based on distinct clinical and laboratory features. Utilizing a variety of techniques including candidate gene studies, the use of genotyping arrays such as Immunochip, and genome wide association studies (GWAS), both human leukocyte antigen (HLA) and non-HLA susceptibility loci associated with JIA have been described. Several of these polymorphisms (e.g. HLA class II, PTPN22, STAT4) are shared with other common autoimmune conditions; other novel polymorphisms that have been identified may be unique to JIA. Associations with oligoarticular and RF-negative polyarticular JIA are the best characterized. A strong association between HLA DRB1:11:03/04 and DRB1:08:01, and a protective effect of DRB1:15:01 have been described. HLA DPB1:02:01 has also been associated with oligoarticular and RF-negative polyarticular JIA. Besides PTPN22, STAT4 and PTPN2 variants, IL2, IL2RA, IL2RB, as well as IL6 and IL6R loci also harbor variants associated with oligoarticular and RF-negative polyarticular JIA. RF-positive polyarticular JIA is associated with many of the shared epitope encoding HLA DRB1 alleles, as well as PTPN22, STAT4 and TNFAIP3 variants. ERA is associated with HLA B27. Most other associations between JIA categories and HLA or non-HLA variants need confirmation. The formation of International Consortia to ascertain and analyze large cohorts of JIA categories, validation of reported findings in independent cohorts, and functional studies will enhance our understanding of the genetic underpinnings of JIA.

Objective. To assess the specific contribution of memory impairment to employment status in persons with systemic lupus erythematosus (SLE). Methods. A total of 832 patients with SLE were surveyed and data collected on demographics, SLE symptoms and activity, health status, depression, medications, health resource utilization, and current employment status. Participants underwent screening for memory impairment and based on their scores were categorized to 3 levels of memory function: intact, mild-moderate impairment, and severe impairment. Employment status was compared across impairment levels using multivariate logistic regression, adjusting for sociodemographic characteristics (i.e., age, sex, race, education, and marital status), employment status at year of diagnosis, disease activity, disease duration, and depression. Results. In the intact memory function group, 54.2% were employed, versus 40.6% in the mild-moderate impairment group and 31.0% in the severe impairment group. In the intact memory function group, 29.2% were unable to work, versus 40.6% in the mild-moderate impairment group and 56.3% in the severe impairment group. After multivariate adjustment, increasing levels of memory impairment predicted a decreased likelihood of being employed: odds ratio (OR) 0.70, 95% confidence interval (95% CI) 0.48 -1.02 for the mild-moderate impairment group and OR 0.57, 95% CI 0.32-1.00 for the severe impairment group. Participants with memory impairment were more likely to report being unable to work: OR 1.36, 95% CI 0.90 -2.04 for the mild-moderate impairment group, and OR 1.99, 95% CI 1.12-3.55 for the severe impairment group. These findings were statistically significant only in the severe impairment groups. Conclusion. The findings suggest that severe memory impairment is an important factor associated with employment status in persons with SLE.