Childhood-onset SLE Research Articles

Improving routine mental health screening for depression and anxiety in a paediatric lupus clinic: a quality improvement initiative for enhanced mental healthcare

BackgroundMental health (MH) conditions are prevalent in adolescents with childhood-onset SLE (cSLE). Early identification is crucial in preventing poor patient outcomes; however, MH screening rates remain low.Local problemFrom July 2021–January...

Favourable humoral but reduced cellular immune response to COVID-19 mRNA BNT162b2 vaccine in patients with childhood-onset systemic lupus erythematosus

ObjectiveTo evaluate both humoral and cellular immune responses to the COVID-19 messenger RNA (mRNA; BNT162b2) vaccine in patients with childhood-onset SLE (cSLE) compared with healthy controls and patient controls (kidney...

Active implementation of low disease activity state as a treatment endpoint in childhood-onset systemic lupus erythematosus in routine practice is both feasible and associated with better outcomes

IntroductionTreat-to-target (T2T) strategies aim to facilitate tight disease control to improve outcomes. No previous studies evaluated prospectively the feasibility and impact of the T2T strategy in routine practice in childhood-onset SLE (cSLE).MethodsAdolescents and young adults (AYA) with cSLE were recruited for T2T implementation from a large tertiary centre over a period of 6 months and followed up at least twice over a prospective period of 12 months.ResultsDuring Oct 2022–April 2023, 135/162 (83.3%) AYA with cSLE had disease scores evaluated at their routine appointment to enable inclusion in the study, and 122/135 (91.2%) had their disease assessed, and a suitable treatment target agreed and documented at each routine clinical appointment over the 12 months prospective follow-up. T2T strategy led to improved disease control at 12 months: more AYA with cSLE achieved clinical remission off steroids (4.1% vs. 10.7%, P = 0.048), or minimum childhood-lupus low disease activity (cLLDAS) (81.9% vs. 91.8%, P = 0.022). Achieving minimum cLLDAS for longer than 3 months was associated with reduced damage accrual (HR = 1.7; 95%CI = 1.1–2.5; P < 0.0001) at 12 months.ConclusionT2T strategy implementation was achievable and associated with improved cSLE control. Spending at least 3/12 months in cLLDAS led to less damage accumulation.Key Points• This is the first large prospective study in AYA with cSLE to evaluate the impact of active T2T implementation in routine practice.• T2T strategies were feasible to implement in 122/135 (91.2%) AYA with cSLE in routine practice.• The T2T approach was associated with improved disease control and decreased damage accrual at 12 months.

Barriers and facilitators to medical care retention for pediatric systemic lupus erythematosus in South Africa: a qualitative study

BackgroundSystemic lupus erythematosus (SLE) is a life-threatening, chronic, autoimmune disease requiring long term subspecialty care due to its complex and chronic nature. Childhood-onset SLE (cSLE) is more severe than adult-onset, and the cSLE population in South Africa has been reported to have an even higher risk than patients elsewhere. Therefore, it is critical to promptly diagnose, treat, and manage cSLE. In this paper, we aim to describe and evaluate barriers and enablers of appropriate long-term care of cSLE South Africa from the perspective of caregivers (parents or family members).MethodsCaregivers (n = 22) were recruited through pediatric and adult rheumatology clinics. Individuals were eligible if they cared for youth (≤ 19 years) who were diagnosed with cSLE and satisfied at least four of the eleven ACR SLE classification criteria. Individual in-depth, semi-structured interviews were conducted between January 2014 and December 2014, and explored barriers to and facilitators of ongoing chronic care for cSLE. Data were analyzed using applied thematic analysis.ResultsFour barriers to chronic care engagement and retention were identified: knowledge gap, financial burdens, social stigma of SLE, and complexity of the South African medical system. Additionally, we found three facilitators: patient and caregiver education, robust support system for the caregiver, and financial support for the caregiver and patient.ConclusionThese findings highlight multiple, intersecting barriers to routine longitudinal care for cSLE in South Africa and suggest there might be a group of diagnosed children who don’t receive follow-up care and are subject to loss to follow-up. cSLE requires ongoing treatment and care; thus, the different barriers may interact and compound over time with each follow-up visit. South African cSLE patients are at high risk for poor outcomes. South African care teams should work to overcome these barriers and place attention on the facilitators to improve care retention for these patients and create a model for other less resourced settings.

#896 Long-term renal outcome in childhood-onset lupus nephritis

Abstract Background and Aims Lupus nephritis (LN) is a common and severe complication of childhood-onset SLE. Little is known about the long-term renal outcome of childhood-onset LN. We investigated predictive factors of end-stage kidney disease (ESKD) and chronic kidney disease (CKD) in a paediatric cohort of patients with LN. Method This is a retrospective multicentre study including patients with childhood-onset (&amp;lt;18 years) biopsy-proven LN. Clinical features were analysed at disease onset, time of kidney biopsy, 12 and 24 months after biopsy, and last follow-up. We investigated the prognostic significance of clinical, laboratory and histologic characteristics on the incidence of ESKD and CKD stage 3-5. Results We included 73 patients with a median age of 14 years (IQR 11.5-17) . At the time of kidney biopsy, most subjects displayed an aggressive disease: 2 (3%) children required haemodialysis, 27 (38%) had an estimated glomerular filtration rate (eGFR) &amp;lt;60 mL/min/1.73 m2. The overall median eGFR was 70 mL/min/1.73 m2 (IQR 43-96) . Moreover, the median proteinuria was 4 g/24 h (IQR 1.35-7.41) , with 41 (59%) children displaying a nephrotic range proteinuria (i.e., &amp;gt;3 g/24 h) . The median follow-up was 13.3 years (IQR 4.7-25.4) . A total of 10 patients (13.7%) reached ESKD, the majority of whom (12%) within 10 years from kidney biopsy; during the subsequent follow-up the incidence of ESKD stabilised (Fig. 1). At last follow-up, around 50% of patients displayed an eGFR &amp;lt;90 mL/min/1.73 m2. A younger age at LN onset, a lower eGFR and central nervous system involvement at the time of kidney biopsy were identified as predictors of ESKD by a univariable Cox regression model (Table 1). The same features were significantly associated with the occurrence of CKD stage 3-5 at last follow-up at a univariable logistic regression analysis. Conclusion LN often presents with severe kidney function impairment and aggressive systemic involvement in children. In our cohort, whose follow-up was among the longest reported in the literature, 12% of patients reached ESKD within 10 years from kidney biopsy. Significant predictors of poor kidney outcome in the long term were a younger age at LN onset, a lower eGFR and the presence of neurological manifestations at the time of kidney biopsy.

Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

ObjectiveTo derive childhood-onset SLE (cSLE) specific remission definitions for future treat-to-target (T2T) trials, observational studies, and clinical practice. MethodsThe cSLE International T2T Task Force conducted Delphi surveys exploring paediatric perspectives on adult-onset SLE remission targets. A modified nominal group technique was used to discuss, refine, and agree on the cSLE remission target criteria. ResultsThe Task Force proposed two definitions of remission: ‘cSLE clinical remission on steroids (cCR)’ and ‘cSLE clinical remission off steroids (cCR-0)’. The common criteria are: (1) Clinical-SLEDAI-2 K = 0; (2) PGA score < 0.5 (0–3 scale); (4) stable antimalarials, immunosuppressive, and biologic therapy (changes due to side-effects, adherence, weight, or when building up to target dose allowed). Criterion (3) in cCR is the prednisolone dose ≤0.1 mg/kg/day (maximum 5 mg/day), whereas in cCR-0 it is zero. ConclusionscSLE definitions of remission have been proposed, maintaining sufficient alignment with the adult-SLE definition to facilitate life-course research.

DNA methylation of IFI44L as a potential blood biomarker for childhood-onset systemic lupus erythematosus

BackgroundIFN-induced protein 44-like (IFI44L) promoter methylation has been demonstrated to serve as an effective blood diagnostic biomarker for adult-onset SLE. However, its utility as a diagnostic marker for childhood-onset SLE (cSLE) remains to be verified.MethodsInitially, we conducted a differential analysis of gene methylation and mRNA expression patterns in cSLE whole blood samples obtained from the public GEO database to determine IFI44L gene expression and assess the methylation status at its CpG sites. Subsequently, we collected clinical whole blood samples from 49 cSLE patients and 12 healthy children, employing an HRM-qPCR-based IFI44L methylation detection technique to evaluate its diagnostic efficacy in pediatric clinical practice.ResultsA total of 26 hypomethylated, highly expressed genes in cSLE were identified by intersecting differentially expressed genes (DEGs) and differentially methylation genes (DMGs). GO enrichment analysis for these 26 genes indicated a robust association with type I IFN. Among the overlapping genes, IFI44L exhibited the most pronounced differential expression and methylation. In subsequent clinical validation experiments, IFI44L methylation was confirmed as an effective blood-based diagnostic biomarker for cSLE, achieving an AUC of 0.867, a sensitivity of 0.753, and a specificity of 1.000.ConclusionsIFI44L methylation is a promising blood biomarker for cSLE.ImpactIFI44L promoter methylation was reported to serve as a highly sensitive and specific diagnostic marker for adult-onset SLE. However, the diagnostic efficacy of IFI44L in childhood-onset SLE (cSLE) still remains to be confirmed. In this study, we utilized bioinformatics analysis and conducted clinical experiments to demonstrate that IFI44L methylation can also serve as a promising blood biomarker for cSLE. The findings of this study can facilitate the diagnosis of cSLE and broaden our understanding of its molecular mechanisms, with a particular focus on those related to type I interferons.

Childhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series review

BackgroundIncreased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis.MethodA retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol.ResultsOf the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min–max) SLEDAI-2 K scores were 9 (0–38), median (min–max) SLICC/ACR-DI (SDI) score were 1 (1–5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma.ConclusionEstimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series.

Expansion of extrafollicular B and T cell subsets in childhood-onset systemic lupus erythematosus.

Most childhood-onset SLE patients (cSLE) develop lupus nephritis (cLN), but only a small proportion achieve complete response to current therapies. The prognosis of children with LN and end-stage renal disease is particularly dire. Mortality rates within the first five years of renal replacement therapy may reach 22%. Thus, there is urgent need to decipher and target immune mechanisms that drive cLN. Despite the clear role of autoantibody production in SLE, targeted B cell therapies such as rituximab (anti-CD20) and belimumab (anti-BAFF) have shown only modest efficacy in cLN. While many studies have linked dysregulation of germinal center formation to SLE pathogenesis, other work supports a role for extrafollicular B cell activation in generation of pathogenic antibody secreting cells. However, whether extrafollicular B cell subsets and their T cell collaborators play a role in specific organ involvement in cLN and/or track with disease activity remains unknown. We analyzed high-dimensional mass cytometry and gene expression data from 24 treatment naïve cSLE patients at the time of diagnosis and longitudinally, applying novel computational tools to identify abnormalities associated with clinical manifestations (cLN) and disease activity (SLEDAI). cSLE patients have an extrafollicular B cell expansion signature, with increased frequency of i) DN2, ii) Bnd2, iii) plasmablasts, and iv) peripheral T helper cells. Most importantly, we discovered that this extrafollicular signature correlates with disease activity in cLN, supporting extrafollicular T/B interactions as a mechanism underlying pediatric renal pathogenesis. This study integrates established and emerging themes of extrafollicular B cell involvement in SLE by providing evidence for extrafollicular B and peripheral T helper cell expansion, along with elevated type 1 IFN activation, in a homogeneous cohort of treatment-naïve cSLE patients, a point at which they should display the most extreme state of their immune dysregulation.

Evaluation of belimumab in treatment of Chinese childhood-onset systemic lupus erythematosus: a prospective analysis from a multicentre study.

The aim of this study is to identify whether low lupus disease activity status (LLDAS) and clinical remission (CR) of belimumab plus standard of care (SoC) therapy are achievable goals in childhood-onset SLE (cSLE). This multicentre, one arm pre-post intervention study was conducted at 15 centres in China. The primary end point was to describe the proportion of patients who achieved LLDAS and CR after 3, 6 and 12 months after treatment with belimumab plus SoC therapy. A multiple regression model was used to impute missing data. A Poisson regression model was used to calculate the effect of belimumab treatment on the reduced risk of serious diseases and the incidence of new damage. A total of 193 (92.2% female) with active cSLE from 15 centres were included. At 3, 6 and 12 months, the proportion of LLDAS (CR) was 12.4% (1.0%), 25.6% (4.5%) and 70.3% (29.7%), respectively. The mean SELENA-SLEDAI score decreased from 11.0 at baseline to 3.7, 2.9 and 1.7 at 3, 6 and 12 months. At baseline, all patients received steroids at a mean (s.d.) prednisone equivalent dose of 31.0 (18.2) mg/day, which decreased to 19.4 (10.8) mg/day at month 3, 12.6 (7.2) mg/day at month 6 and 6.7 (5.3) mg/day at month 12. The symptoms and immunological indicators were also significantly improved. This is the first and largest sample size prospective clinical intervention study of cSLE patients treated with belimumab in China. LLDAS and CR were attainable treat-to-target of belimumab plus SoC therapy in cSLE.

Treatment patterns in paediatric and adult patients with SLE: a retrospective claims database study in the USA

ObjectiveTo assess real-world treatment regimens and patterns in childhood-onset SLE (cSLE) and adult-onset SLE (aSLE) cohorts, including similarities in treatments, duration of use and adherence.MethodsThis retrospective study utilised data from...

Usefulness of the lupus low disease activity state as a treatment target in childhood-onset SLE

ObjectiveTreat-to-target (T2T) strategies are advocated to improve prognosis in childhood-onset SLE (cSLE). Proposed T2T states include SLEDAI score of <4 (SLEDAI-LD), limited corticosteroid use (low-CS), and lupus low disease activity...

Targeted multiomics in childhood-onset SLE reveal distinct biological phenotypes associated with disease activity: results from an explorative study

ObjectiveTo combine targeted transcriptomic and proteomic data in an unsupervised hierarchical clustering method to stratify patients with childhood-onset SLE (cSLE) into similar biological phenotypes, and study the immunological cellular landscape...

The value of 2019 EULAR/ACR classification criteria in predicting lupus nephritis in childhood-onset systemic lupus erythematosus.

We investigated the role of European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for the prediction of LN among children with SLE. The data of the patients with childhood-onset SLE diagnosed based on 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were retrospectively evaluated. Based on 2019 EULAR/ACR classification criteria, the scoring was done at the time of renal biopsy. Fifty-two patients (12 with LN, 40 without LN) were included. The mean score was higher in patients with LN than those without (30.8±6.14, 19.8±7.76, respectively, p=0.000). The score value had indicative value for LN (area under curve [AUC]:0.863±0.055, cut-off value:22.5, p=0.000). Lymphocyte counts had a predictive value for LN (cut-off value:905/mm3, AUC:0.688±0.087, p=0.042). The score was positively associated with SLE disease activity index (SLEDAI) and activity index (r=0.879, p=0.000; r=0.811, p=0.001, respectively). There were significant negative associations between score value and GFR (r=-0.582, p=0.047). The patients with renal flare had higher the mean score than those of without renal flare (35±2/25.4±5.57, respectively, p=0.019). The EULAR/ACR criteria score could reflect the activity of disease and severity of nephritis in childhood-onset SLE. A point of 22.5 as score value might be an indicator for LN. During scoring, it should be taken into account that lymphopenia might guide the prediction of LN.

Barriers to vaccination in immunocompromised children: A needs assessment in children with childhood-onset SLE and inflammatory bowel disease.

Vaccination of immunocompromised children (ICC) remains suboptimal. Needs assessment surveys were administered to patients and caregivers during routine ambulatory visits to the rheumatology and gastroenterology clinics at Nationwide Children's Hospital (NCH) from January 1 through August 31, 2018, and to community primary care physicians (PCPs) at their monthly meeting and electronically. Completed surveys were received for 57 patients (31 with childhood-onset systemic lupus erythematosus (c-SLE) and 26 with inflammatory bowel disease (IBD)) and 30 PCPs. Of the patient cohort, 93% (n = 53) felt their PCP was well informed about vaccines and 84% (n = 47) received vaccinations from either their PCP or local health department. Two patient surveys noted concerns of vaccine safety. Among the 30 responses completed by PCPs 50% (n = 15) preferred to provide all vaccines themselves, however, only 40% (n = 12) of PCPs felt "very confident" when providing vaccines to ICC. Further, 83% (n = 25) did not stock the 23-valent pneumococcal vaccine and only 27% (n = 8) routinely recommended vaccination of household contacts. Our study found a discordance between parent and PCP comfort in vaccinating ICC, highlighting an important barrier to vaccination in this patient population. In our cohort of patients, vaccine hesitancy was not a barrier to vaccination.

Genetics of osteonecrosis in children and adults with systemic lupus erythematosus.

Genetics plays an important role in SLE risk, as well as osteonecrosis (ON), a significant and often debilitating complication of SLE. We aimed to identify genetic risk loci for ON in people with childhood-onset (cSLE) and adult-onset (aSLE) SLE. We enrolled participants from two tertiary care centres who met classification criteria for SLE. Participants had prospectively collected clinical data and were genotyped on a multiethnic array. Un-genotyped single nucleotide polymorphisms (SNPs) were imputed, and ancestry was inferred using principal components (PCs). Our outcome was symptomatic ON confirmed by imaging. We completed time-to-ON and logistic regression of ON genome-wide association studies (GWASs) with covariates for sex, age of SLE diagnosis, five PCs for ancestry, corticosteroid use and selected SLE manifestations. We conducted separate analyses for cSLE and aSLE and meta-analysed results using inverse-variance weighting. Genome-wide significance was P < 5 × 10-8. The study included 940 participants with SLE, 87% female and 56% with cSLE. ON was present in 7.6% (n = 71). Median age of SLE diagnosis was 16.9 years (interquartile range [IQR]: 13.5, 29.3), with median follow-up of 8.0 years (IQR: 4.2, 15.7). Meta-GWAS of cSLE and aSLE time-to-ON of 4 431 911 SNPs identified a significant Chr.2 SNP, rs34118383 (minor allele frequency = 0.18), intronic to WIPF1 (hazard ratio = 3.2 [95% CI: 2.2, 4.8]; P = 1.0 × 10-8). We identified an intronic WIPF1 variant associated with a 3.2 times increased hazard for ON (95% CI: 2.2, 4.8; P = 1.0 × 10-8) during SLE follow-up, independent of corticosteroid exposure. The effect of the SNP on time-to-ON was similar in cSLE and aSLE. This novel discovery represents a potential ON risk locus. Our results warrant replication.

Cognitive Performance in Patients with Systemic Lupus Erythematosus Using the Ped-ANAM

Computerized batteries have been widely used to investigate cognitive impairment (CI) in patients with SLE. The aim of this study was to evaluate the cognitive performance of patients with SLE in relation to healthy controls using the Pediatric Automated Neuropsychological Assessment Metrics (Ped-ANAM) battery. In addition, we aimed to examine differences in Ped-ANAM scores according to age of disease onset, presence of disease activity, and disease damage. We included 201 consecutive adult-onset (aSLE) and childhood-onset SLE (cSLE) patients who were being followed at the hospital's rheumatology outpatient clinic and 177 healthy controls. We applied the percentage of correct answers on the Ped-ANAM subtests and the Performance Validity Index (PVI) metric and correlated them with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus Erythematosus Damage Index (SDI). Then, we established their relationships with neuropsychiatric systemic lupus erythematosus (NPSLE). We observed CI in a total of 38 (18.9%) SLE patients and 8 (4.5%) healthy controls (p < 0.001). CI was observed in eight (19.5%) cSLE patients and 32 (20%) aSLE patients (p = 0.8175). Individual analysis of the aSLE subtests showed a significant difference in all subtests compared to healthy controls; the greatest differences were in matching to sample (p < 0.001) and memory search ( p < 0.001). In the cSLE group, we observed a difference in the code substitution subtests (p = 0.0065) compared to the healthy controls. In the evaluation of clinical outcomes, disease activity was significantly correlated with CI in cSLE (r = 0.33; p = 0.042) and aSLE (r = 0.40; p = 0.001). We also observed an association between disease activity and neuropsychiatric manifestations (p = 0.0012) in aSLE. In conclusion, we determined that cognitive dysfunction, mainly in memory and attention, was more prevalent in patients with SLE. In both the cSLE and aSLE groups, disease activity was associated with worse cognitive function. This is the first study to use the Ped-ANAM in Brazil. Longitudinal studies are necessary to determine how the Ped-ANAM will perform over time.

Serum IFNα2 levels are associated with disease activity and outperform IFN-I gene signature in a longitudinal childhood-onset SLE cohort.

To study the association of serum IFNα2 levels measured by ultrasensitive single-molecule array (Simoa) and the IFN-I gene signature (IGS) with disease activity and determine whether these assays can mark disease activity states in a longitudinal cohort of childhood-onset SLE (cSLE) patients. Serum IFNα2 levels were measured in 338 samples from 48 cSLE patients and 67 healthy controls using an IFNα Simoa assay. Five-gene IGS was measured by RT-PCR in paired whole blood samples. Disease activity was measured by clinical SELENA-SLEDAI and BILAG-2004. Low disease activity was defined by Low Lupus Disease Activity State (LLDAS) and flares were characterized by SELENA-SLEDAI flare index. Analysis was performed using linear mixed models. A clear positive correlation was present between serum IFNα2 levels and the IGS (r = 0.78, P < 0.0001). Serum IFNα2 levels and IGS showed the same significant negative trend in the first 3 years after diagnosis. In this timeframe, mean baseline serum IFNα2 levels decreased by 55.1% (Δ 201 fg/ml, P < 0.001) to a mean value of 164 fg/ml, which was below the calculated threshold of 219.4 fg/ml that discriminated between patients and healthy controls. In the linear mixed model, serum IFNα2 levels were significantly associated with both cSELENA-SLEDAI and BILAG-2004, while the IGS did not show this association. Both IFN-I assays were able to characterize LLDAS and disease flare in receiver operating characteristic analysis. Serum IFNα2 levels measured by Simoa technology are associated with disease activity scores and characterize disease activity states in cSLE.

Health disparities in outcomes of pediatric systemic lupus erythematosus.

Healthcare disparities exist throughout the United States, and disparities in healthcare delivery are responsible for a substantial portion of preventable morbidity and mortality. SLE disproportionately affects racial and ethnic minoritized groups, including Blacks, Hispanics, and Asians/Pacific Islanders. Specifically, Black females have a 3 to 4-fold increased risk of developing SLE than White females. Population studies funded through the Centers for Disease Control have examined variations in disease outcomes among the different populations around the United States. For example, studies have shown that lupus nephritis, anti-phospholipid syndrome, and thrombocytopenia are more likely to affect racial and ethnic minorities than Whites. In addition, the Center for Disease Control WONDER (Wide-ranging Online Data for Epidemiologic Research) database found SLE was the seventh leading cause of death for all women aged 15–25 years and the fifth leading cause of death for African American and Hispanic females. From these studies, we know SLE primarily affects racial and ethnic minorities, but we do not know why these groups are at increased risk of developing the disease or have worse outcomes. By examining the underlying mechanisms of health disparities within our patient populations and mitigation strategies, we will further understand and provide better treatment for our patients. This review will discuss current research related to health disparities and health outcomes in childhood-onset SLE (cSLE).

Standardized nailfold capillaroscopy in children with rheumatic diseases: a worldwide study.

To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD.