A single-center model for implementation of SLEDAI documentation adherence in childhood-onset systemic lupus erythematosus (cSLE).

BACKGROUND: Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune disease with variable disease expression but noted association with significant disease-related damage, morbidity, and mortality. The European Alliance of Associations for Rheumatology (EULAR) recommends routine monitoring of SLE through validated disease activity and chronicity indices, including the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Despite this, physician adherence with SLEDAI documentation remains elusive at various academic institutions. The aim of our study was to determine baseline SLEDAI documentation rates at our center and assess the change in adherence in SLEDAI documentation rate with electronic clinical decision support (CDS) reminders facilitated through the electronic medical record (EMR) over a 2-year period. METHODS: All SLE encounters over a 24-month period at a pediatric academic center were reviewed in order to obtain baseline SLEDAI documentation percentages. Physicians subsequently received monthly email reminders, initiated at month 4 of project initiation, with subsequent CDS reminder 13 months after project initiation prompted by anti-dsDNA lab result. Chart review was repeated continuously for each provider, and SLEDAI documentation rates were emailed to each provider monthly. Physicians completed a post-intervention survey regarding barriers to SLEDAI documentation at the end of the study. RESULTS: A total of 1980 SLE encounters were reviewed for this study. Baseline SLEDAI documentation rates were 10%. Following the introduction of monthly emails reminding physicians to document SLEDAI, rates increased to 55%. After the initiation of electronic in-basket reminders prompted by lab results, rates increased to 60%. Noted barriers to documentation were cited to be forgetfulness (67%) and lack of time (33%). CONCLUSION: Our study demonstrates that monthly email reminders as well as EMR-mediated electronic in-basket reminders increased SLEDAI documentation rates at an academic center. Noted barriers to documentation were reported to be forgetfulness (67%) and lack of time (33%).

Secukinumab Therapy in Refractory Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA), the most common chronic rheumatologic condition in childhood, remains a cause of significant morbidity, particularly in those with spondyloarthropathy, including psoriatic arthritis (PsA) and enthesitis-related arthritis (ERA). While secukinumab was recently approved for the treatment of children and adolescents with ERA and PsA, there is limited published data on its use in JIA, particularly in refractory cases, despite its efficacy in the treatment of adult arthritis. We aim to examine the use of this therapy in JIA in a single pediatric rheumatology center. A retrospective chart review was performed and 10 JIA patients who received treatment with secukinumab were identified. Data extracted included disease activity, patient demographics, comorbidities, medications, and laboratory data. Seven ERA, 2 PsA, and 1 poly JIA patient were treated with secukinumab at our center between April 2011 and July 2021. These patients had notably resistant disease, with a mean disease-modifying antirheumatic drug (DMARD) failure rate of 3.8. One hundred percent of patients who underwent magnetic resonance imaging (MRI) after being on at least 3 months of secukinumab therapy demonstrated improvement in their MRI findings. One patient developed a flare of uveitis while on secukinumab therapy, with no other adverse events recorded in our patients. Secukinumab therapy was recently approved for children and adolescents with ERA and PsA, and may offer an efficacious option given its demonstrated improvement in imaging and joint examination, as well as qualitative reports of pain, even in those who have failed other therapies. However, caution may be warranted in those with a history of uveitis and warrants further study.

COVID-19 after rituximab therapy in cSLE patients.

Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune disease associated with significant morbidity and mortality. Rituximab is a B-cell depleting therapy utilized in the treatment of SLE. In adults, rituximab has been associated with increased risk of adverse outcomes in patients who develop coronavirus disease 2019 (COVID-19). We aimed to assess the impact of prior rituximab treatment on clinical outcomes from Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection in children with SLE. To describe the impact of rituximab on outcomes from SARS-CoV-2 infection, we conducted a retrospective study of pediatric SLE patients in our center diagnosed with COVID-19 who had previously received rituximab between February 2019 and October 2022. Patients' clinical characteristics, disease activity, and outcomes were assessed. Of the eight subjects assessed, five required hospitalizations for COVID-19, four required ICU admission, and two were seen in the emergency department for their symptoms. One patient ultimately expired from her illness. The median time between rituximab administration and COVID-19 diagnosis was 3 months. We assessed the clinical outcomes, including the need of ICU admission and fatal outcome, of COVID-19 in our cSLE patient population after rituximab administration. Approximately 60% of our patients required hospitalization for their illness, and seven out of eight patients required healthcare utilization to include hospitalization and/or emergency department visits.

Impact of follow-up adherence on disease activity in childhood-onset systemic lupus erythematosus (cSLE).

BACKGROUND/PURPOSE: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, with a potential for significant disease damage, morbidity, and mortality. In comparison to the adult population, childhood-onset SLE (cSLE) tends to be more aggressive given the higher preponderance of renal and neuropsychiatric disease and increased disease activity. There is a paucity of literature examining relationship between disease activity, rheumatology follow-up visits, and health care utilization. The objective of this study is to determine whether adherence with outpatient clinic visits would affect disease activity in patients with childhood-onset systemic lupus erythematosus (cSLE). METHODS: 41 children <18 years of age at time of diagnosis with SLE who met Systemic Lupus International Collaborative Clinics (SLICC) criteria and not evaluated in clinic within the previous 120-day period were identified as eligible for inclusion. Patients were continuously searched between December 2021 and July 2022 for eligibility evaluation. Through retrospective chart review, we assessed disease activity (SLE Disease Activity Index) at the last clinic visit. The patients were stratified into two cohorts of lower and higher disease activity, with SLE disease activity index (SLEDAI) ≤ 3 and SLEDAI ≥ 4, respectively. Descriptive statistics and Willcox Rank Sum (numerical variables) and Fisher's test (categorical variables) were used to compare these two groups. RESULTS: Clinical, epidemiological, and serological data were compared between the two groups, with observed statistically significant differences to include current use of high dose prednisone associated with higher SLEDAI scores (p = 0.019). In nonparametric analysis, time to follow-up (p < 0.001), hospitalizations (p = 0.017), and Emergency Department visits (ED) (p < 0.001) were found to be associated with higher SLEDAI scores. CONCLUSION: Our findings suggest that cSLE patients with higher disease activity are at risk for increased health care utilization with respect to ED visits as well as hospitalizations in the setting of follow-up nonadherence. While further studies are required to enhance our understanding of this association, this links the importance of disease-related outcome and routine outpatient visits in this particularly vulnerable patient population.

Clinical Determinants of Childhood Onset Systemic Lupus Erythematosus among Early and Peri-Adolescent Age Groups.

Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is associated with significant morbidity and mortality. SLE disproportionately affects women and minorities. Childhood-onset SLE (cSLE) in particular tends to be more aggressive than adult-onset SLE. Despite substantial improvements in the treatment of cSLE, there is significant variability in treatment responses and long-term outcomes. Furthermore, there is a paucity of studies involving cSLE, and in particular, cSLE among different age groups. The aim of this study was to test the hypothesis that an early-onset cSLE cohort would demonstrate unique characteristics with distinctive clinical and laboratory features at disease onset. We specifically investigated whether clinical, epidemiological, or serological factors are differentially associated with early- and late-onset cSLE. This could have direct impact on clinical management with the goal of improving outcomes and quality of life for children with SLE. Methods: Our study was conducted at a large tertiary center. We included 213 subjects seen at our pediatric rheumatology clinic aged 4−17 years. Epidemiologic, clinical phenotype, disease severity, serology, treatment, and outcome data were compared between subjects with cSLE onset prior to 10 years of age (early-onset disease, n = 43) and those with cSLE onset greater than 10 years of age (peri-adolescent disease, n = 170). We compared clinical features between early- and peri-adolescent onset cSLE in order to investigate the association between age at disease onset of cSLE and clinical disease expression and outcomes. Results: Of the 213 subjects with cSLE in our study, 43 subjects had early-onset disease (age 2 to ≤9 years) and 170 patients had peri-adolescent onset disease. We found that early-onset cSLE was associated with a higher prevalence of positive anti-dsDNA antibody at cSLE diagnosis, higher anti-dsDNA antibody titer at cSLE diagnosis, rash, and azathioprine use (p < 0.001, p = 0.004, p = 0.011, and p = 0.008, respectively). In contrast, we found that peri-adolescent onset cSLE (≥10 years of age) was associated with worse disease activity (SLEDAI range 0−24) (p < 0.001), higher SLICC at diagnosis (p < 0.001), as well as a higher rate of mycophenolate mofetil and hydroxychloroquine use (p = 0.003 and p < 0.001, respectively). There were no significant differences in the prevalence of neuropsychiatric symptoms or the development of Class IV/Class V lupus nephritis between the early-onset and peri-adolescent groups.

Presentation of SLE after COVID vaccination in a pediatric patient.

BACKGROUND: The outbreak of severe acute respiratory syndrome coronavirus 2 has had an enormous impact on global health. Vaccination remains one of the most effective interventions for disease prevention. Clinically significant vaccine side effects are uncommon, though autoimmune-mediated disease occurs in a small percentage of vaccine recipients. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is associated with significant morbidity and mortality. Childhood-onset SLE tends to have more severe disease manifestations than adult-onset SLE. In adults, there are a few reported cases of SLE developing soon after coronavirus disease 2019 (COVID-19) mRNA vaccination. CASE PRESENTATION: A 14-year-old previously healthy male developed laboratory and clinical evidence of SLE, including maculopapular malar rash, arthritis, pleuritic chest pain, and class V (membranous) lupus nephritis, 2 days after his third dose of the Pfizer-BioNTech COVID-19 vaccine. The patient's symptoms improved after initiation of prednisone and mycophenolate mofetil. We also summarize eleven prior case reports describing SLE after COVID-19 vaccine in adults. CONCLUSION: To our knowledge, this is the first reported pediatric patient with new onset SLE following COVID-19 mRNA vaccination. While potential mechanistic links exist between COVID-19 vaccination and SLE development, additional studies are necessary to elucidate the exact nature of this relationship.

Quality of life measures and physical activity in childhood systemic lupus erythematosus.

Childhood systemic lupus erythematosus (cSLE) is a life-long disease with significant morbidity and mortality, and with associated significant impact on health-related quality of life (HRQOL). Previous literature supports that physical activity has positive impact on HRQOL in patients with chronic diseases, including cSLE. We sought to describe the physical activity of our patients with cSLE and determine the relationship between physical activity, SLE activity, treatment modalities and HRQOL in cSLE. Children ≤18 years of age with cSLE and their parents were enrolled and completed corresponding child and parent Simple Measure of Impact of Lupus Erythematosus in Youngsters© reports (cSMILEY© and pSMILEY©, respectively), and the Physical Activity Questionnaire for Children (PAQ-C) or Adolescents (PAQ-A). Through retrospective chart review, we assessed the SLE Disease Activity Index (SLEDAI) using the SLEDAI-2K assessment tool. Descriptive statistics as well as Pearson's correlation coefficients were performed with the data obtained. Forty-four children and their parents were enrolled; clinical data, SMILEY© and PAQ-C or PAQ-A scores of cSLE subjects were evaluated. The most frequently reported physical activity modality was walking (61.3%), with mean frequency of 3.7 ± 1.8 days a week, and a median of 3.5 days a week. Although there was no correlation noted between treatment modalities and PAQ-C/PAQ-A, there was weak correlation between SLEDAI and PAQ-C/PAQ-A (Pearson correlation= 0.2, ρ = 0.1, p = 0.9, n = 44). There was a weak correlation between SMILEY total score and PAQ [cSMILEY© and PAQ-C/PAQ-A combined cohorts (Pearson correlation = 0.2, ρ = 0.3, p = 0.07, n = 44), and modest correlation between pSMILEY© scores and PAQ-C/PAQ-A combined cohorts (Pearson correlation = 0.3, ρ = 0.3, p = 0.05, n = 44)]. Our study emphasizes the need for larger samples to understand the prognostic value of activity levels and the extent to which increasing physical activity might be linked to improvements in HRQOL in this vulnerable population.