A 5-year follow-up of pulmonary function tests in childhood-onset systemic lupus erythematosus: a single-center retrospective study.

SIGNIFICANCE: Pulmonary involvement in childhood-onset systemic lupus erythematosus (cSLE), contributes to significant morbidity and mortality. Manifestations include chronic interstitial pneumonitis, pneumonia, pleuritis, alveolar hemorrhage, and shrinking lung syndrome. However, many patients can be asymptomatic from a respiratory standpoint and still have pulmonary function test (PFT) abnormalities. Our aim is to describe PFT abnormalities in patients with cSLE. METHODS: We completed a retrospective review of 42 patients with cSLE followed at our center. These patients were at least 6 years old (so they could complete PFTs). We collected data from July 2015 to July 2020. RESULTS: Out of the 42 patients, 10 (23.8%) had abnormal PFTs. These 10 patients had a mean age at diagnosis of 13 ± 2.9 years. Nine were female. One-fifth (20%) self-identified as Hispanic, 20% as Asian, 10% as Black or African American, and the remaining 50% as "Other." Of the 10, 3 had restrictive disease only, 3 with diffusion impairment only, and 4 with both restrictive lung disease and diffusion impairment. Patients with restrictive patterns had a mean total lung capacity (TLC) of 72.5 ± 5.8 throughout the study period. The average diffusing capacity for carbon monoxide corrected for hemoglobin (DsbHb) among patients with diffusion limitation during the study period was 64.8 ± 8.3. CONCLUSIONS: The most common PFT abnormalities seen in patients with cSLE are alterations in diffusing capacity as well as restrictive lung disease.

Longitudinal program evaluation of an inter-institutional mentorship network for pediatric rheumatology using a quality improvement framework.

Background: The American College of Rheumatology (ACR)/Childhood Arthritis and Rheumatology Research Alliance (CARRA) Mentoring Interest Group (AMIGO) is an inter-institutional mentorship program launched to target mentorship gaps within pediatric rheumatology. Initial program evaluation indicated increased mentorship access. Given the small size of the pediatric rheumatology workforce, maintaining a consistent supply of mentors was a potential threat to the longevity of the network. Our aims were to: (i) describe the sustainability of AMIGO over the period 2011-2018, (ii) highlight ongoing benefits to participants, and (iii) describe challenges in the maintenance of a mentorship network. Methods: A mixed-methods approach centered on a quality improvement framework was used to report on process and outcomes measures associated with AMIGO annual cycles. Results: US and Canada Pediatric rheumatology workforce surveys identified 504 possible participants during the time period. As of fall 2018, 331 unique individuals had participated in AMIGO as a mentee, mentor or both for a program response rate of 66% (331/504). Survey of mentees indicated high satisfaction with impact on general career development, research/scholarship and work-life balance. Mentors indicated increased sense of connection to the community and satisfaction with helping mentees despite minimal perceived benefit to their academic portfolios. Based on AMIGO's success, a counterpart program, Creating Adult Rheumatology Mentorship in Academia (CARMA), was launched in 2018. Conclusions: Despite the challenges of a limited workforce, AMIGO continues to provide consistent access to mentorship opportunities for the pediatric rheumatology community. This experience can inform approaches to mentorship gaps in other academic subspecialties.

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.

Acute respiratory distress syndrome and COVID-19 in a child with systemic lupus erythematosus.

We report a case of COVID-19 in a pediatric patient with systemic lupus erythematosus (SLE), who presented with respiratory distress marked by increased work of breathing and low oxygen saturation. Lab tests confirmed COVID-19, and showed lymphocytopenia and elevated markers of inflammation and coagulopathy. Chest X-ray showed bilateral mid-lung opacities, and the patient required intubation early in his disease course. Imaging and clinical findings were consistent with acute respiratory distress syndrome (ARDS) with inflammation. The patient was treated with different combinations of antivirals (hydroxychloroquine and remdesivir), cytokine inhibitors (anakinra and tocilizumab), glucocorticoids (hydrocortisone and methylprednisolone), and an anticoagulant (enoxaparin). Inflammatory markers decreased before clinical improvement in lung aeration. This case highlights the potential for pediatric patients with SLE to present with COVID-19 similar to the clinical presentation described in adults.

Making Decisions About Stopping Medicines for Well-Controlled Juvenile Idiopathic Arthritis: A Mixed-Methods Study of Patients and Caregivers.

OBJECTIVE: Improved treatments for juvenile idiopathic arthritis (JIA) have increased remission rates. We conducted this study to investigate how patients and caregivers make decisions about stopping medications when JIA is inactive. METHODS: We performed a mixed-methods study of caregivers and patients affected by JIA, recruited through social media and flyers, and selected by purposive sampling. Participants discussed their experiences with JIA, medications, and decision-making through recorded telephone interviews. Of 44 interviewees, 20 were patients (50% ages <18 years), and 24 were caregivers (50% caring for children ages ≤10 years). We evaluated characteristics associated with high levels of reported concerns about JIA or medicines using Fisher's exact testing. RESULTS: Decisions about stopping medicines were informed by competing risks between disease activity and treatment. Participants who expressed more concerns about JIA were more likely to report disease-related complications (P = 0.002) and more motivated to continue treatment. However, participants expressing more concern about medicines were more likely to report treatment-related complications (P = 0.04) and felt more compelled to stop treatment. Additionally, participants considered how JIA or treatments facilitated or interfered with their sense of normalcy and safety, expressed feelings of guilt and regret about previous or potential adverse events, and reflected on uncertainty and unpredictability of future harms. Decision-making was also informed by trust in rheumatologists and other information sources (e.g., family and online support groups). CONCLUSION: When deciding whether to stop medicines whenever JIA is inactive, patients and caregivers weigh competing risks between disease activity and treatment. Based on our results, we suggest specific approaches for clinicians to perform shared decision-making regarding stopping medicines for JIA.

Multi-pronged approach to enhance education of children and adolescents with lupus, caregivers, and healthcare providers in New Jersey: Needs assessment, evaluation, and development of educational materials.

BACKGROUND: Childhood Systemic Lupus Erythematosus (cSLE) patients are younger at diagnosis and have a more severe disease course compared to adult onset SLE patients and develop significant complications related to disease and or immunosuppression. Moreover, female and minority populations experience higher rates of cSLE, with African American, Afro-Caribbean, and Hispanic populations being at greatest risk and having poor prognosis. METHODS: The Pediatric Alliance for Lupus initiative addressed the dearth in education and resources in a multi-stage process. First, we conducted a need assessment identifying knowledge gaps among healthcare providers (HCPs), and resources needed to care for cSLE patients and their families. Second, we educated HCPs about the diagnosis and treatment of cSLE by Continuing Medical Education (CME) sessions/webinars (presented here). Third, HCPs participated in a Quality Improvement (QI) program on cSLE approved by the American Board of Pediatrics Maintenance of Certification Part 4. Finally, patients and caregivers were educated through the development of appropriate, culturally and linguistically sensitive cSLE resources. PAL disseminated materials among HCPs and the community to improve the awareness of the availability of these materials. RESULTS: According to results from the statewide needs assessment (representative of every county throughout NJ), HCPs face significant challenges in providing care to cSLE patients and their families, in part due to the multi-systemic nature of the autoimmune disease. CONCLUSION: Based on this need, we developed educational sessions, with pre-post comparison data showing a significant increase in knowledge after HCP education. The 15 different materials developed as part of the endeavor is a major contribution to the cSLE community, HCPs and pediatric rheumatologists. Resources are available in multiple formats (PDF and web pages), and are accessible on the National Resource Center on Lupus, the latest web site of the Lupus Foundation of American that houses materials for SLE patients, their families, schools, HCPs, and the community at large.Improving cSLE knowledge will empower the children and adolescents and families by increasing their self-efficacy; and positively impact key health outcomes (transition readiness and HRQOL) that are not optimally addressed with current medical treatment alone.

The use of interactive distraction technique to manage gagging during impression taking in children: a single-blind, randomised controlled trial.

PURPOSE: To evaluate the effect of interactive distraction technique on the severity of gagging and success of impression taking in children between age 5 and 10 years. METHODS: This single blind, randomised controlled trial recruited 48 children aged 5-10 years requiring maxillary impressions. Selected children were randomly allocated to one of the two groups: (1) Test group, use of interactive distraction, and (2) Control group, no use of interactive distraction, during impression taking. Dental anxiety was scored using Facial Image Scale (FIS) before and after impression taking. Gagging-related Impression Success Scale (GISS) and Gagging Severity Index (GSI) were recorded by a blinded assessor. Nonparametric tests were used to compare the variables between the groups. RESULTS: Maxillary impressions were successfully recorded in all children in the Test group; either with no gag (87.5%) or with gag (12.5%). In 33.3% control children impressions could not be recorded. Statistically significant, strong association was found between the interactive distraction and GISS (p = 0.003, Cramer's V = 0.32). Mean GSI and post-impression FIS scores were significantly lesser in the Test group (p = 0.029 and p = 0.048, respectively). Significant increase in the mean FIS scores was observed after recording impression in the Control group. Post-impression FIS showed significant positive correlation with GSI (Test: p = 0.003, Control: p = 0.009) and significant inverse correlation with GISS (Test: p = 0.003, Control: p = 0.01). CONCLUSION: Interactive distraction technique used in this study is a simple, non-invasive and cost-effective method to effectively manage gagging and successfully record a maxillary impression in children between age 5 and 10 years.

Epidermal Neurite Density in Skin Biopsies From Patients With Juvenile Fibromyalgia.

OBJECTIVE: Fibromyalgia (FM) is defined by idiopathic, chronic, widespread musculoskeletal pain. In adults with FM, a metaanalysis of lower-leg skin biopsy demonstrated 45% pooled prevalence of abnormally low epidermal neurite density (END). END < 5th centile of the normal distribution is the consensus diagnostic threshold for small-fiber neuropathy. However, the clinical significance of END findings in FM is unknown. Here, we examine the prevalence of small-fiber pathology in juvenile FM, which has not been studied previously. METHODS: We screened 21 patients aged 13-20 years with FM diagnosed by pediatric rheumatologists. Fifteen meeting the American College of Rheumatology criteria (modified for juvenile FM) underwent lower-leg measurements of END and completed validated questionnaires assessing pain, functional disability, and dysautonomia symptoms. The primary outcome was proportion of FM patients with END < 5th centile of age/sex/race-based laboratory norms. Cases were systematically matched by ethnicity, race, sex, and age to a group of previously biopsied healthy adolescents with selection blinded to biopsy results. All 23 controls matching demographic criteria were included. RESULTS: Among biopsied juvenile FM patients, 53% (8/15) had END < 5th centile vs 4% (1/23) of healthy controls (P < 0.001). Mean patient END was 273/mm2 skin surface (95% CI 198-389) vs 413/mm2 (95% CI 359-467, P < 0.001). As expected, patients with FM reported more functional disability, dysautonomia, and pain than healthy controls. CONCLUSION: Abnormal END reduction is common in adolescents with FM, with similar prevalence in adults with FM. More studies are needed to fully characterize the significance of low END in FM and to elucidate the clinical implications of these findings.

A North American perspective of content and quality of websites in the English language on childhood-onset lupus erythematosus.

Objective The objective of this article is to examine the quality, content, and readability of information and resources in the English language and accessible on the internet by pediatric patients with systemic lupus erythematosus (SLE) and their families in North America. Methods Keywords relevant to SLE were generated by an undergraduate student, a first-year medical student, and a third-year pediatric resident, and a search was conducted across five commonly used search engines. Quality of information found was evaluated independently by an undergraduate student, a graduate student, a first-year medical student, and a third-year pediatric resident using the DISCERN tool. Two pediatric rheumatologists assessed website accuracy and completeness. Readability of websites was determined using the Flesch-Kincaid grade level and Reading Ease score. Results Out of 2000 websites generated in the search, only 34 unique websites met inclusion criteria. Only 16 of these websites had DISCERN scores above 50% (fair quality). Overall quality of website information was fair with mean ±standard deviation (SD) DISCERN quality score of 44 ± 7 (range: 30-56). Only nine websites of 34 had DISCERN scores above 50 (>66%, indicating greater quality) and were further assessed for completeness. Flesch-Kincaid grade level was 11 ± 1 (mean±SD) and reading ease score was 39 ± 10 (mean±SD, range of 11-61). Conclusion Our study highlights the need for more complete, readable information regarding the unique needs of pediatric patients with childhood-onset SLE and their families.

Clinical characteristics of children with membranous lupus nephritis: the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry.

Objective The objective of this article is to describe and compare clinical features, treatment, and renal outcomes of children with membranous lupus nephritis (MLN), through analysis of a national multicenter registry. Methods Patients with pediatric systemic lupus erythematosus (SLE) and MLN from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were included. Demographic, disease and medication-related data were collected between 2010 and 2014 from 59 CARRA Legacy Registry sites. Results A total of 132 individuals had MLN, either in isolation or in combination with proliferative LN. Seventy-four patients had pure MLN. The proportion of patients with daily corticosteroid treatment was similar among groups (96%, 91%, and 96%, for class III+V, IV+V, and V, respectively, p = 0.67). Proportion of individuals exposed to any disease-modifying antirheumatic drug (DMARD) or biologic was similar among the three groups (83%, 91%, 95% for class III+V, IV+V, and V, respectively, p = 0.189). Proportion of patients with decreased glomerular filtration rate (less than 90 ml/min/1.73 m2) was significantly different among groups (4%, 38%, and 4%, for class III+V, IV+V, and V, respectively, p < 0.0001). Conclusion This is the largest reported cohort of children with MLN. More research is needed to understand treatment practices for pediatric MLN, particularly decisions related to pharmacologic treatment of pure MLN. More work is also needed to identify prognostic factors and predictors of outcome for pediatric MLN. Future observational studies will be a first step toward understanding and formulating a standardized approach to treatment of pediatric membranous LN and allowing for the initiation of prospective comparative effectiveness studies and interventional trials.

Relationship between health-related quality of life, disease activity and disease damage in a prospective international multicenter cohort of childhood onset systemic lupus erythematosus patients.

Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits. Physicians assessed disease activity and damage. The multinational cohort ( n = 467) had relatively low disease activity and damage. Patient and parent HRQOL scores were significantly correlated. Asian and European patients had the highest HRQOL, while South and North American patients had lower HRQOL scores. Renal, CNS, skin and musculoskeletal systems exhibited the highest levels of damage. North and South American and Asian patients were more likely to have disease damage and activity scores above median values, compared with Europeans. Asians were more likely to use cyclophosphamide/rituximab. Female gender, high disease activity and damage, non-White ethnicity, and use of cyclophosphamide and/rituximab were related to lower HRQOL. HRQOL domain scores continue to emphasize that SLE has widespread impact on all aspects of children's and parents' lives.

Unrecognized Celiac Disease in Children Presenting for Rheumatology Evaluation.

BACKGROUND AND OBJECTIVES: Current clinical guidelines do not consider patients with rheumatic conditions to be at high risk for celiac disease (CD) despite numerous reported associations between the two in adults and children. The objective of this study was to evaluate the prevalence of CD among patients presenting for pediatric rheumatology evaluation. METHODS: A total of 2125 patients presenting for initial evaluation by the Division of Pediatric Rheumatology at the Hospital for Special Surgery between June 2006 and December 2013 were screened for CD as a part of the standard initial serologic evaluation. The charts of these patients were evaluated retrospectively at the end of this period. RESULTS: 36 patients (30 girls, 6 boys, mean age 9.4 ± 4.3 years, range 2-16 years) received a diagnosis of CD after serologic testing and evaluation by pediatric gastroenterology. Eight additional patients with known diagnoses of CD presented during this time period. The total prevalence of CD over this 6.5-year period was 2.0%. The most common presenting complaints among patients diagnosed with CD were myalgias, arthralgias, and rash. Less frequently, patients reported gastrointestinal complaints including abdominal pain, nausea, and diarrhea. All patients reported improvement or complete resolution of their musculoskeletal symptoms after initiation of a gluten-free diet. CONCLUSIONS: This study identified 36 new cases of CD among children presenting for rheumatology evaluation, for an overall prevalence rate of 2.0%. The majority of patients who ultimately received a diagnosis of CD presented with extraintestinal manifestations. These results underscore the importance of screening children presenting for rheumatology evaluation for CD.

Luminescence properties of Eu(3)(+)-doped Na3Gd(PO4)2 red-emitting nanophosphors for LEDs.

Eu(3+)-doped Na3Gd(PO4)2 phosphors were synthesized by the modified citrate gel combustion technique. From the XRD pattern, structure of the compound was found to be orthorhombic with a particle size of 10-20nm. The emission spectra revealed that the Na3Gd(PO4)2:Eu(3+) phosphors can be excited effectively by 394nm pump wavelength. From the concentration dependent photoluminescence studies, the optimum concentration for efficient luminescence of Eu(3+) ions in Na3Gd(PO4)2 phosphor has been found to be 1.5mol%. Decay curves of the (5)D0 level of Eu(3+) ions were recorded by monitoring the emission at 596nm corresponding to (5)D0→(7)F1 transition and are found to be single exponential for all the concentrations. The chromaticity properties for different concentrations of Na3Gd(PO4)2:Eu(3+) nanophosphors were calculated from emission spectra and analyzed in the frame work of CIE color diagram.

Choosing Wisely: the American College of Rheumatology's Top 5 for pediatric rheumatology.

OBJECTIVE: To create a pediatric rheumatology Top 5 list as part of the American Board of Internal Medicine Foundation's Choosing Wisely campaign. METHODS: Delphi surveys of a core group of representative pediatric rheumatology providers from across North America generated candidate Top 5 items. Items with high content agreement and perceived to be of prevalent use and of high impact were included in a survey of all American College of Rheumatology (ACR) members who identified themselves as providing care to pediatric patients. Items with the highest ratings were subjected to literature review and further evaluation. RESULTS: A total of 121 candidate items were proposed in the initial Delphi survey and were reduced to 28 items in subsequent surveys. These 28 items were sent to 1,198 rheumatology providers who care for pediatric patients, and 397 (33%) responded. Based upon survey data and literature review, the Top 5 items were identified. These items focused on testing for antinuclear antibodies, autoantibody panels, Lyme disease, methotrexate toxicity monitoring, and use of routine radiographs. CONCLUSION: The ACR pediatric rheumatology Top 5 is one of the first pediatric subspecialty-specific Choosing Wisely Top 5 lists and provides an opportunity for patients and providers to discuss appropriate use of health care in pediatric rheumatology.

Spectroscopic investigation and optical characterization of Eu3+ ions in K-Nb-Si glasses.

This paper reports on the effect of concentration of Eu(3+) ions in K2O-Nb2O5-SiO2-Eu2O3 (KNbSiEu) glasses prepared by the melt quenching technique. By using the Judd-Ofelt (JO) theory, the intensity parameters Ωλ (λ=2, 4, 6) have been determined from the absorption and emission spectra of Eu(3+) ions under different constraints. The radiative properties of some of the excited states of Eu(3+) ions have been calculated. The decay curves of (5)D0 level exhibited single exponential for all the Eu(3+) ions concentrations. From these results, it is suggested that the strong red emission at 616 nm corresponding to the (5)D0→(7)F2 transition could be used for the development of optical display devices.

Optical and luminescent properties of Sm³+ doped tellurite glasses.

Glasses with chemical composition of (62-x) TeO(2)+25 ZnO+8 K(2)O+5 CaO+x Sm(2)O(3) (TZKCSmx; x=0.1, 0.5, 1.0, 1.5 mol%) were prepared by melt quenching technique. The absorption spectrum was recorded in the UV-visible and NIR regions. The oscillator strengths of absorption bands were obtained by measuring the area under the bands. Judd-Ofelt analysis has been carried out to estimate the host dependent J-O intensity Ω(λ) (λ=2, 4, 6) parameters by least squares fitting approach. Photoluminescence spectra recorded in the visible region revealed intense green, orange and red emission bands in all the glasses, corresponding to the (4)G(5/2)→(6)H(5/2), (4)G(5/2)→(6)H(7/2) and (4)G(5/2)→(6)H(9/2) transitions respectively. From the emission spectra and J-O intensity parameters, various radiative parameters were calculated from the excited (4)G(5/2) to the lower lying (6)H(J) (J=5/2, 7/2, 9/2, 11/2) multiplet. Quenching of luminescence with the increase of Sm(3+) ions concentration has been observed. Decay times of excited (4)G(5/2) state decrease with the increase of the Sm(3+) ions concentration. The energy transfer mechanism that leads to the quenching of (4)G(5/2) state lifetime has been discussed. Inokuti-Hirayama (I-H) model was used to evaluate various energy transfer parameters, which are the qualitative indicators for the interaction among Sm(3+) ions.