Objectives The aims of this study are (1) to characterize factors influencing self-management behaviors and quality of life in adolescent and young adult (AYA) patients with childhood-onset systemic lupus erythematosus (cSLE) and (2) to identify barriers and facilitators of treatment adherence via focus groups. Methods AYAs with cSLE ages 12–24 years and primary caregivers of the adolescents participated in this study. Recruitment occurred during pediatric rheumatology clinic visits at a Midwestern children's hospital or the hospital's cSLE active clinic registry. Information about disease severity was obtained from patient health records. Pain and fatigue questionnaires were administered. Descriptive statistics were used to analyze data. Results Thirty-one AYA patients and caregivers participated in six focus groups. Ten major themes emerged from sessions; four were expressed both by the AYA and caregiver groups: knowledge deficits about cSLE, symptoms limiting daily function, specifically mood and cognition/learning, barriers and facilitators of adherence, and worry about the future. Themes unique to AYA participants included symptoms limiting daily functioning—pain/fatigue, self-care and management, impact on personal relationships, and health care provider communication/relationship. For caregiver groups unique themes included need for school advocacy, disruption of family schedule, and sense of normalcy for their adolescent. Conclusion AYAs with cSLE face a lifelong disease characterized by pervasive pain, fatigue, organ damage, isolation—social and/or physical—and psycho-socioeducational challenges. This study confirmed that continued psychosocial support, health information education, adherence interventions, and personalized treatment plans are necessary to increase self-management and autonomy in AYAs with cSLE.

Background: Serum phagocyte-derived alarmins S100A8/9 and S100A12 are considered useful for the assessment of inflammatory diseases. Our study evaluated the use of S100 proteins in a pediatric clinical setting for estimating disease activity and supporting diagnosis.Methods: Patients (n = 136) who had S100 proteins tested as part of clinical care were included in this study and relevant information obtained from the medical record: C-reactive protein (CRP), disease activity status (inactive: = 0 joint; active: > 0 active joint), systemic symptoms in systemic JIA (sJIA), and symptoms of flare of other autoinflammatory and fever syndromes. Patients were categorized as: sJIA, non-systemic JIA (nsJIA), other defined autoinflammatory syndromes (AID) and systemic undifferentiated recurring fever syndromes (SURFS).Results: Patients with sJIA (n = 21) had significantly higher levels of S100A8/9 and S100A12 compared to patients with nsJIA (n = 49), other AIDs (n = 8) or SURFS (n = 14) (all p < 0.0001). Compared to CRP [area under the receiver operating characteristics curve (AUC) = 0.7], S100 proteins were superior in differentiating sJIA from AID and SURFS [AUC = 0.9]. S100A8/9 and S100A12 levels were not associated with disease activity in nsJIA, AID or SURFS. S100A8/9 and S100A12 levels were significantly higher in active sJIA compared to inactive (p = 0.0002 and p = 0.0002 respectively).

Objective Hepatitis B virus (HBV) infection remains a significant public health challenge, particularly for immunocompromised patients. Our aim was to evaluate the serologic immunity in immunocompromised rheumatology and inflammatory bowel disease (IBD) patients, assess factors for serologic non-immunity and evaluate their response to one HBV booster dose. Methods Immunocompromised rheumatology and IBD patients with completed HBV screening were identified. A chart review was performed to collect demographics, clinical information, baseline HBV serology results, and serologic response to booster vaccination. Serologic nonimmunity was defined as a negative/indeterminate hepatitis B surface antibody (anti-HBs) level. Results Among 580 patients, 71% were non-immune. The highest portion of non-immune patients were 11-18 years old (p 0.004). There was no significant difference between immune and non-immune patients with regards to diagnosis (p 0.342), age at diagnosis (p 0.639), duration of treatment (p 0.069) or type of medications (p 0.080). Sixty-two percent of those who received a booster vaccine were re-screened, and most (68%) seroconverted. In those 18 years or older, only half seroconverted. Conclusion Results of this study support the benefit of HBV screening in immunosuppressed patients. Beginning at age 11 years most patients lacked serologic immunity to HBV. Seroconversion for most patients 11-18 years occurred after one booster vaccine. Thus, for immunocompromised patients without recent HBV serologic data, obtaining the HBV serology beginning at age 11 years might be considered. Those 18 years and older were least likely to seroconvert after one booster, indicating that they may benefit from receiving the three-dose HBV vaccine series.

Objective. To compare the diagnostic usefulness of the 2019 European League Against Rheumatism (EULAR)/ American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus (SLE) to that of the 1997 ACR classification criteria for SLE when applied to youths (age ≤21 years) with SLE. Methods. Data were extracted from electronic health records of patients followed at a large academic pediatric hospital. The treating rheumatologist's diagnosis of SLE served as the standard criterion for identifying SLE patients (cases). Controls were patients with juvenile dermatomyositis (DM), juvenile scleroderma, or juvenile systemic sclerosis (SSc). The 2019 EULAR/ACR criteria and the 1997 ACR criteria were tested against the standard criterion. Results. A total of 112 SLE patients ages 2-21 years and 105 controls ages 1-19 years (66% juvenile DM, 34% juvenile scleroderma or juvenile SSc) were available for analysis. The 2019 EULAR/ACR criteria had significantly higher sensitivity (85% versus 72%; P = 0.023) and similar specificity (83% versus 87%; P = 0.456) than the 1997 ACR criteria. The mean ± SD 2019 EULAR/ACR classification summary scores were significantly higher among non-White than White patients (22.41 ± 10.04 versus 17.59 ± 9.19; P < 0.01). The sensitivity of the 2019 EULAR/ACR criteria in non-White/White patients was 92%/80% (P = 0.08) versus 83%/64% (P < 0.02) for the 1997 ACR criteria. The sensitivity of the 2019 EULAR/ACR criteria was not affected by age or sex. Conclusion. The 2019 EULAR/ACR criteria efficiently classify youths with SLE, irrespective of age, sex, and race. Compared to the 1997 ACR criteria, the new criteria are significantly more sensitive and similarly specific in youths with SLE.

Introduction: Lupus nephritis (LN) is a common and significant manifestation, affecting 60% of adults and 80% of children with systemic lupus erythematosus, with up to 30% of patients progressing to end stage renal disease. There remains an unmet need for non-invasive markers of disease activity, damage, and response to therapy. In addition, non-invasive biomarkers that predict therapeutic efficacy are needed to enable cost-effective clinical trials of novel agents. Areas covered: This review examines the methodological aspects of urinary proteomics, the role of proteome profiling in identifying promising urinary biomarkers in LN, and the translation of research findings into clinically useful tools in the management of LN. Expert opinion: Targeted and unbiased proteomics have identified several promising urinary biomarkers that predict LN activity, damage (chronicity), and response to therapy. In particular, a combination of biologically plausible urinary biomarkers termed as RAIL (Renal Activity Index for Lupus) has emerged as an excellent predictor of LN activity as well as response to therapy, being able to predict efficacy within 3 months of therapy. If validated in additional large prospective studies, the RAIL biomarkers will transform the care of patients with LN, allowing for a personalized and predictive approach and improved outcomes.