Normative data on lower extremity entheseal tendon thicknesses in healthy children: an ultrasound study correlating age, sex, anthropometry.

OBJECTIVES: This study aims to establish normative data on lower extremity entheseal tendon thicknesses in healthy children and examine correlations with age, gender, and anthropometric measures using musculoskeletal ultrasound. The secondary objective of the study is to investigate the power Doppler properties of entheseal tendons. METHODS: A total of 192 healthy children, aged 5-18 years, participated in this cross-sectional study. Participants underwent detailed physical and ultrasonographic examinations. Entheseal tendon thickness measurements were taken from five specific regions: distal quadriceps tendon (DQT), proximal patellar ligament (PPL), distal patellar ligament (DPL), Achilles tendon (AT), and plantar fascia (PF). Correlations between thicknesses and age, weight, height, and body mass index (BMI) were analyzed. Intra-tendinous vascularity was evaluated using power Doppler. Interobserver and intraobserver agreements were assessed using intraclass correlation coefficients (ICC). RESULTS: Normative data on lower extremity entheseal tendon thicknesses according to age, weight, height, and BMI have been established. Significant positive correlations were found between thicknesses and age, weight, height, and BMI. Weight was identified as the most influential factor, particularly for the DPL and AT. Right side tendons (AT and PF) are statistically thicker. Minimal Doppler activity was detected in 10.6% of the entheseal DQTs in the group of children aged 5-9 years. The study achieved high to excellent interobserver and intraobserver agreement. CONCLUSION: This study examined the ultrasonographic characteristics of lower extremity entheseal tendons in healthy children using B-mode and power Doppler, provided normative data on their thicknesses, and demonstrated significant correlations between thicknesses and age, sex, and anthropometry.

Prevalence of spine pain among Tunisian children and adolescents and related factors.

BACKGROUND: The prevalence of back and neck pain is common in children and adolescents, and in some series the numbers are alarming. Various risk factors have been identified, although some are controversial. OBJECTIVE: To determine the prevalence of neck and back pain in children and adolescents and to investigate the potential association with various risk factors identified in the literature. METHODS: We established a questionnaire targeting parents of children and adolescents aged between 6 and 18 years old in Tunisia. The recruitment of participants was done online using the Google Forms application. The questionnaire was divided into 2 parts: Part one collected the sociodemographics characteristics of the participants : age, gender, body mass index (BMI), exposure to passive smoking, the practice of a physical activity, puberty status and age at puberty if applicable, type and weight of the schoolbag, mean daily time spent on electronic devices, type of school the child attends (private/public), mode of transport from home to school, parental history of neck and/or back pain (mid or low back pain (LBP)), posture of the sitting position of the child, and finally whether the child reports neck/ back pain. The second part was aimed at parents whose child reported neck and/or back pain. We asked about the weekly frequency of neck/back pain, school absenteeism due to neck/back pain, whether it prevented the child from practicing physical activity and, finally, whether the child had ever seen a doctor/chiropractor/physiotherapist for their neck/back pain. RESULTS: Eighty-eight children (45 females, 43 males) were enrolled. Mean age was 11.9 ± 3.8 years [6-18]. Mean BMI was 18.8 ± 4.2 [15.8-35.5]. Thirty-four (38.6%) were pubescent. Twenty-five (28.4%) children were exposed to passive smoking. Parental history of spine pain was found in 58% of cases. A poor sitting position was noted in n = 49 (55.7%). Mean daily screen time was 88.3 ± 75.56 min [0-360]. Prevalence of spine pain was 44% (n = 39) distributed as follows: neck pain (n = 21, 23.8%), mid back pain (n = 15, 17%), LBP (n = 26, 29.5%), neck, mid back and low back pain (n = 4, 4.5%) Professional help seeking for spine pain in children was reported by 15 participants (25.3%). Among them, 20.3% visited a physician and 5% consulted a chiropractor or physiotherapist. A significant correlation was found between spine pain and age (p = 0.006) and BMI (p = 0.006). A significant association was found between LBP and exposure to passive smoking, puberty status, type of school bag and poor posture. A positive parental history of spine pain was significantly associated with the presence of spine pain in their children with p = 0.053 (neck pain), p = 0.013 (back pain) and p < 0.00 (LBP) respectively. A significant association was found between the presence of spine pain and school absenteeism, participation in sports, consultation with a doctor or physiotherapist/chiropractor (p < 0.0001 respectively). CONCLUSION: The prevalence of spinal pain was frequent in our series. A positive parental history of spinal pain, a bad posture while sitting, passive smoking, use of backpack, higher age and higher BMI were potential associated factors.

Recurrent pericarditis and interleukin (IL)-1 inhibitors.

Recurrent pericarditis (RP) is defined by the European Society of Cardiology (ESC) as an instance of acute pericarditis (AP) that occurs at least 4-6 weeks after the resolution of a previous episode of the same ailment. To mitigate the risk of RP, it is advised to administer accurate and prolonged pharmacological treatment for both the initial AP and subsequent RP. ESC guidelines recommend commencing treatment for any single episode of AP, including those that contribute to RP, with non-steroidal anti-inflammatory drugs (NSAIDs) in conjunction with colchicine for several months, often followed by gradual tapering. If there is an inadequate response, corticosteroids (CS) may be introduced cautiously. However, in a minority of cases, even when NSAIDs, colchicine, and CS are administered together at the highest recommended dosages, they may prove ineffective. In such instances, treatment with immunosuppressive drugs or biologics is advised. Among biologics, interleukin (IL)-1 inhibitors have been extensively studied, although certain gaps remain. This narrative review delves into the rationale for employing IL-1 inhibitors and presents findings from existing studies regarding their efficacy, tolerability, and safety. Analysis of the literature indicates that there is currently insufficient data to ascertain the true therapeutic role of IL-1 inhibitors in managing and preventing RP. However, theoretically, drugs targeting both IL-1α and IL-1ß may offer superior efficacy compared to those solely targeting IL-1ß due to the significant involvement of both cytokines in inflammation. Further research is warranted to determine the comparative effectiveness of IL-1α and IL-1ß inhibitors.

Childhood-Onset Systemic Lupus Erythematosus (cSLE): An International Perspective.

PURPOSE OF REVIEW: Childhood-onset systemic lupus erythematosus (cSLE) is a severe and potentially life-threatening chronic autoimmune disease. cSLE is more aggressive and has poorer outcomes than adult-onset disease. The global burden of cSLE is poorly understood, with most publications on cSLE originating from high-resourced settings. The reports from less resourced settings indicate high morbidity and mortality in these populations. RECENT FINDINGS: In this article, we review the disparities in global access to rheumatology care and research for patients with cSLE. We highlight recent cSLE advances from all regions of the globe. We describe current obstacles to cSLE clinical care and research in all settings. Finally, we propose a path forward for high quality, equitable and accessible care to individuals with cSLE everywhere. Individuals with cSLE are at risk for morbidity and death, yet patients worldwide face challenges to adequate access to care and research. Sustained, collaborative efforts are needed to create pathways to improve care and outcomes for these patients.

An unusual case of pediatric granulomatosis with polyangiitis complicated by splenic infarction presenting as inflammatory bowel disease.

We describe a case of granulomatosis with polyangiitis (GPA) in a 7-year-old-male who initially presented with symptoms concerning for Inflammatory bowel disease. GPA is a rare, multisystemic necrotizing vasculitis involving small arteries and veins. The clinical presentation can be variable given its multisystemic involvement but more commonly involves the upper and lower airways and kidneys. This case highlights rare gastrointestinal symptoms of GPA, further complicated by an additional unique finding of splenic infarction. We hope to raise awareness for this rare illness to assist in diagnosis and treatment, as timely induction of remission can reduce significant morbidity and mortality in the pediatric population.

The critical role of parents within a Learning Health Network.

Parent members of the Pediatric Rheumatology Care & Outcomes Improvement Network are an integral part of the Learning Health Network's work. Since early in the creation of the network, they have been a part of every Quality Improvement project, committee, and work group and have a role in governance on the Executive and Steering Committees. Members of the Parent Working Group (PWG) have played a role in developing QI measures used in the clinical setting as well as initiatives and projects like the guiding work of Treat-to-Target. The PWG also creates self-management supports, including toolkits for families and patients at all stages of life. This article will discuss how integrating parents as partners in a pediatric Learning Health Network is critical for the quality of care received by children with chronic illnesses and to improving outcomes.

Reliability of a generative artificial intelligence tool for pediatric familial Mediterranean fever: insights from a multicentre expert survey.

BACKGROUND: Artificial intelligence (AI) has become a popular tool for clinical and research use in the medical field. The aim of this study was to evaluate the accuracy and reliability of a generative AI tool on pediatric familial Mediterranean fever (FMF). METHODS: Fifteen questions repeated thrice on pediatric FMF were prompted to the popular generative AI tool Microsoft Copilot with Chat-GPT 4.0. Nine pediatric rheumatology experts rated response accuracy with a blinded mechanism using a Likert-like scale with values from 1 to 5. RESULTS: Median values for overall responses at the initial assessment ranged from 2.00 to 5.00. During the second assessment, median values spanned from 2.00 to 4.00, while for the third assessment, they ranged from 3.00 to 4.00. Intra-rater variability showed poor to moderate agreement (intraclass correlation coefficient range: -0.151 to 0.534). A diminishing level of agreement among experts over time was documented, as highlighted by Krippendorff's alpha coefficient values, ranging from 0.136 (at the first response) to 0.132 (at the second response) to 0.089 (at the third response). Lastly, experts displayed varying levels of trust in AI pre- and post-survey. CONCLUSIONS: AI has promising implications in pediatric rheumatology, including early diagnosis and management optimization, but challenges persist due to uncertain information reliability and the lack of expert validation. Our survey revealed considerable inaccuracies and incompleteness in AI-generated responses regarding FMF, with poor intra- and extra-rater reliability. Human validation remains crucial in managing AI-generated medical information.

Genetic association of antinuclear antibodies with HLA in JIA patients: a Swedish cohort study.

BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is a complex autoimmune disease and the most common chronic rheumatological disease affecting children under the age of 16. The etiology of JIA remains poorly understood, but evidence suggests a significant genetic predisposition. METHODS: We analyzed a Swedish cohort of 329 JIA patients and 728 healthy adult controls using the Illumina OmniExpress array for genotyping. HLA alleles were imputed from GWAS data using the SNP2HLA algorithm. RESULTS: Case-control analysis yielded 12 SNPs with genome-wide significant association to JIA, all located on chromosome 6 within the MHC class II gene region. Notably, the top SNP (rs28421666) was located adjacent to HLA-DQA1 and HLA-DRB1. HLA-DRB1*08:01, HLA-DQA1*04:01, and HLA-DQB1*04:02 were the haplotypes most strongly associated with an increased risk of JIA in the overall cohort. When analyzing disease specific subtypes, these alleles were associated with oligoarthritis and RF-negative polyarthritis. Within the complex linkage disequilibrium of the HLA-DRB1-DQA1-DQB1 haplotype, our analysis suggests that HLA-DRB1*08 might be the primary allele linked to JIA susceptibility. The HLA-DRB1*11 allele group was also independently associated with JIA and specifically enriched in the oligoarthritis patient group. Additionally, our study revealed a significant correlation between antinuclear antibody (ANA) positivity and specific HLA alleles. The ANA-positive JIA group showed stronger associations with the HLA-DRB1-DQA1-DQB1 haplotype, HLA-DRB1*11, and HLA-DPB1*02, suggesting a potential connection between genetic factors and ANA production in JIA. Furthermore, logistic regression analysis reaffirmed the effects of HLA alleles, female sex, and lower age at onset on ANA positivity. CONCLUSIONS: This study identified distinct genetic associations between HLA alleles and JIA subtypes, particularly in ANA-positive patients. These findings contribute to a better understanding of the genetic basis of JIA and provide insights into the genetic control of autoantibody production in ANA-positive JIA patients. This may inform future classification and personalized treatment approaches for JIA, ultimately improving patient outcomes and management of this disease.

Electronic health record modification and dashboard development to improve clinical care in pediatric rheumatology.

Objective: This report describes our experience in electronic health record (EHR) note modification and creation of an external dashboard to create a local learning health system that contributes to quality improvement and patient care within our pediatric rheumatology clinic. Methods: We applied quality improvement methodology to develop a more reliable and accurate system to identify patients with juvenile idiopathic arthritis and track important measures that aide in improving patient care and performance outcomes. From 2019 to 2021, we iteratively modified our outpatient clinic EHR note to include structured data elements to improve longitudinal monitoring. We then validated data transferred to an electronic dashboard external to the EHR and demonstrated utility for identifying an accurate patient population and tracking quality improvement initiatives. Results: Creation of the structured data elements improved the identification of patients with JIA with >99% accuracy and without requiring manual review of the chart. Using the dashboard to monitor performance, we improved documentation of critical disease activity measures that resulted in improvement in those scores across the local population of patients with JIA. The structured data elements also enabled us to automate electronic data transfer to a multicenter learning network registry. Conclusion: The structured data element modifications made to our outpatient EHR note populate a local dashboard that allows real time access to critical information for patient care, population management, and improvement in quality metrics. The collection and monitoring of structured data can be scaled to other quality improvement initiatives in our clinic and shared with other centers.

To switch or to swap? Evidence from a meta-analysis for the best treatment approach in childhood chronic uveitis resistant to the I anti-TNF.

Objective: Since adalimumab approval in childhood chronic non-infectious uveitis (cNIU), the prognosis has been dramatically changed, but the 25 % failed to achieve inactivity. There is not accordance if it is better to switch to another anti-TNF or to swap to another category of biologic. Thus, we aim to summarize evidence regarding the best treatment of cNIU refractory to the first anti-TNF. Methods: A systematic literature review and meta-analysis, according to PRISMA Guidelines, was performed(Jan2000-Aug2023). Studies investigating the efficacy of treatment in cNIU refractory to the first anti-TNF were considered for inclusion. The primary outcome was the improvement of intraocular inflammation according to SUN. A combined estimation of the proportion of children responding to switch or swap and for each drug was performed. Results: 23 articles were eligible, reporting 150 children of whom 109 switched anti-TNF (45 adalimumab, 49 infliximab, 9 golimumab) and 41 swapped to another biologics (31 abatacept, 8 tocilizumab and 1 rituximab). The proportion of responding children was 46 %(95 % CI 23-70) for switch and 38 %(95 % CI 8-73) for swap (χ20.02, p = 0.86). Instead analysing for each drug, the proportion of responding children was the 24 %(95 % CI 2-55) for adalimumab, 43 %(95 % CI 2-80) for abatacept, 79 %(95 % CI 61-93) for infliximab, 56 %(95 % CI 14-95) for golimumab and 96 %(95 % CI 58-100) for tocilizumab. We evaluated a superiority of tocilizumab and infliximab compared to the other drugs(χ2 27.5 p < 0.0001). Conclusion: Although non-conclusive, this meta-analysis suggests that, after the first anti-TNF failure, tocilizumab and infliximab are the best available treatment for the management of cNIU.

Discordant Perception of Disease Activity Between Providers and Adolescents with Systemic Lupus Erythematosus: A Cohort Study.

Discordance in perception of disease activity between adolescent patients with lupus and their providers may influence disease outcomes. We found that patients endorsed higher perceptions of disease activity than providers. Discordance was present at all levels of disease activity, particularly in patients with high activity, nephritis, and/or taking corticosteroids or mycophenolate mofetil.

Real-world application of the pediatric Glucocorticoid Toxicity Index in childhood-onset lupus.

OBJECTIVES: The pediatric Glucocorticoid Toxicity Index (pGTI) is a new, pediatric-specific tool to quantify glucocorticoid (GC)-related morbidity in children. We evaluated the feasibility and construct validity of retrospective pGTI scoring in patients with pediatric-onset systemic lupus erythematosus (pSLE) and identified risk factors for cumulative toxicity. METHODS: We conducted a retrospective cohort study of patients with pSLE treated with GCs at two pediatric centers (1999-2023). GC exposure was estimated using interval-averaged oral prednisone-equivalent dose and cumulative prednisone-equivalent dose. We scored change in GC toxicity every 6 months (±2) using a modified pGTI including 7 of 10 domains. We calculated the Cumulative Worsening Score (CWS), a continuous summation of toxicity accrued. Mixed effects linear regression was used to identify factors associated with CWS. RESULTS: There were 126 patients with pSLE, including 88 with nephritis, with a median of 6 visits/patient. Nearly half (47 %) experienced toxicity in the Blood Pressure domain. Other common toxicities were mood disturbance (25 %), followed by increased body mass index (BMI), striae, and sleep disturbance (21 % each). Decreased growth velocity was observed in 18 %. There was modest correlation between cumulative GC dose and CWS (rho 0.3; p < 0.01). Greater cumulative toxicity was associated with younger age, elevated BMI, and rituximab use at the time of GC initiation, albeit indications for the latter were not captured. CONCLUSIONS: Patients with pSLE experience a high burden of GC toxicity, particularly related to blood pressure, BMI, sleep, and growth. Standardized, pediatric-specific GC toxicity assessment is feasible in real-world settings and can facilitate evaluation of strategies to reduce morbidity in children requiring chronic GC treatment.

Autosomal Recessive Infantile Hyaline Fibromatosis Identified Using Artificial Intelligence-Assisted Rapid Whole Genome Sequencing: A Rare, Multisystemic, Hereditary Disorder.

Infantile hyaline fibromatosis syndrome (HFS) is an ultra-rare genetic condition characterized by the deposition of hyaline material in the skin, muscle, and viscera. Potential complications include debilitating joint contractures, coarse facial features, recurrent infections, failure to thrive, and death. Here, we present the case of a six-month-old infant with a history of painful extremity contractures, global developmental delay, neck hemangioma, and feeding intolerance presenting to our institution with abdominal distension. The multi-systemic, rapidly progressing, severe nature of her symptoms prompted consultation with inpatient pediatric genetics. Per their recommendation, rapid whole-genome sequencing (rWGS) was done with Fabric GEM®-assisted artificial intelligence (Fabric Genomics, Oakland, California, United States) at Rady Children's Hospital Institute for Genomic Medicine (San Diego, California, United States), revealing homozygous pathogenic variant c.652T>C; P.Cys218Arg in the ANTXR2 gene consistent with HFS. This case was significant not only for its rarity, but also its early manifestation of symptoms, wide range of affected body systems, and severity of symptoms, which together present a fascinating diagnostic dilemma for future clinicians that should be taken into consideration. It also highlights the increasing utility of AI-assisted rWGS as a diagnostic tool for medically complex patients with unknown multisystemic hereditary conditions.

The emerging paradigm in pediatric rheumatology: harnessing the power of artificial intelligence.

Artificial intelligence algorithms, with roots extending into the past but experiencing a resurgence and evolution in recent years due to their superiority over traditional methods and contributions to human capabilities, have begun to make their presence felt in the field of pediatric rheumatology. In the ever-evolving realm of pediatric rheumatology, there have been incremental advancements supported by artificial intelligence in understanding and stratifying diseases, developing biomarkers, refining visual analyses, and facilitating individualized treatment approaches. However, like in many other domains, these strides have yet to gain clinical applicability and validation, and ethical issues remain unresolved. Furthermore, mastering different and novel terminologies appears challenging for clinicians. This review aims to provide a comprehensive overview of the current literature, categorizing algorithms and their applications, thus offering a fresh perspective on the nascent relationship between pediatric rheumatology and artificial intelligence, highlighting both its advancements and constraints.

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration. CONCLUSIONS: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests. WHAT IS KNOWN: • During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring. WHAT IS NEW: • There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. • This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests.

The cultural differences in transition assessment: Evaluation of TRANSITION-Q and STARx scales on Turkish rheumatic patients.

Objectives: This study aimed to assess the readiness of our patient population for the transfer to adult care and the applicability of the TRANSITION-Q and STARx scales to the Turkish adolescent patient population. Patients and methods: A total of 153 patients (92 males, 61 females; mean age: 15.5±1.9 years; range, 12 to 18 years) were included in the study between September 15, 2021, and December 15, 2021. The patients were divided into two groups according to age groups: 12 to 15 years old and 16 to 18 years old. The patients were also divided into four groups according to their diagnosis: connective tissue diseases, juvenile idiopathic arthritis, vasculitis, and autoinflammatory diseases. The TRANSITION-Q and STARx scales were administered face-to-face by a nurse and a doctor. The transition readiness of the patients was evaluated according to their scores. Results: Sixty-nine (45%) patients were in the 12 to 15 age group, and 84 (55%) were in the 16 to 18 age group. Eight-four (54.9%) patients had juvenile idiopathic arthritis, 47 (30.7%) patients had an autoinflammatory disease, 14 (9.2%) patients had vasculitis, and eight (5.2%) patients had a connective tissue disease. There was no significant difference in the scale scores according to disease groups and sexes in both scales. Considering the age of the patients, the mean scores of the patients in the 16 to 18 age group were found to be significantly higher compared to the 12 to 15 age group for both the TRANSITION-Q (74.3±13.3 vs. 65.4±9.6, p<0.001) and STARx scales (51.8±8.1 vs. 44.8±9.1, p<0.001). Cronbach's alpha score was 0.71 for the STARx scale and 0.79 for the TRANSITION-Q scale. Conclusion: TRANSITION-Q and STARx scales could guide the Turkish patient population in determining the pretransition needs of patients in planning individualized transition processes.

Telemedicine Usability and Satisfaction among Pediatric Rheumatology Patients and their Caregivers during COVID-19 Pandemic.

Background: The 2019 coronavirus disease pandemic opened an opportunity to explore the role of telemedicine in pediatric rheumatology clinic as well as patient satisfaction with virtual visits. Objective: To determine the usability and satisfaction rate of telemedicine among pediatric rheumatology patients and their caregivers. Method: A cross-sectional online survey was conducted among patients and caregivers consulting via telemedicine at a pediatric rheumatology clinic of University of the Philippines - Philippine General Hospital (UP - PGH), a tertiary government hospital. Collected data included socio-demographics and the validated Telehealth Usability Questionnaire (TUQ). Results: There were 39 (55.7%) patients and 31 (44.3%) primary caregivers included in the study. Across all usability factors, the response of primary caregivers did not significantly differ from those of patients. The average scores across all questions for both patients and primary caregivers were 5.96±1.19 and 6.04±1.34, respectively. This showed a high level of agreement that they were highly satisfied with telemedicine experience. Among the different usability factors, questions related to usefulness obtained the highest mean score for both patients (6.11±1.17) and primary caregivers (6.12±1.38). While the lowest mean score was observed on questions related to reliability, 5.65±1.33 for patients and 5.89±1.31 for primary caregivers. Conclusion: Pediatric rheumatology patients as well as their caregivers are generally highly satisfied with telemedicine during this time of pandemic. With high patient and caregiver satisfaction, telemedicine could be an option for ambulatory patient care even after pandemic.

Effective diagnosis of sacroiliitis in children: evaluating magnetic resonance imaging with the Canadian scoring system.

OBJECTIVES: To reveal the differences by comparing the magnetic resonance imaging (MRI) findings of patients with clinically symptomatic sacroiliitis with those undergoing sacroiliac imaging for other reasons using the Canadian Spondyloarthritis Research Consortium-Sacroiliac Inflammatory Scoring System (SPARCC-SIS). METHODS: In this retrospective single-center study, sacroiliac MRIs performed between 2017 and 2023 were evaluated from the database. The SPARCC-SIS scoring system is used to evaluate and grade the inflammation of the sacroiliac joints. Mild inflammation is indicated by a score below 24, moderate by a score of 24-48, and severe by a score above 49. Additionally, structural defects of the sacroiliac joint, such as erosion, sclerosis, and ankylosis, were observed. After MRI evaluation, clinically symptomatic (group 1) and non-symptomatic (group 2) patients were divided into two groups. The clinical and laboratory findings of the patients and MRI findings were compared. The patient's age, gender, clinical information from hospital records, acute phase reactants (APRs), and the presence of the Human Leukocyte Antigen (HLA-B27) gene (if applicable) were thoroughly recorded. RESULTS: One hundred thirty-six children who performed sacroiliac MRI for any indication were included in the study. The APRs positivity, presence of HLA-B27, and SPARCC scoring system were significantly higher in 24 patients with clinical sacroiliitis (group 1) than in 112 patients without sacroiliitis (group 2). In our study, the most common MRI findings in children were bone marrow edema, capsulitis, synovitis, and erosion, while chronic structural changes such as sclerosis and ankylosing were rare. CONCLUSION: In this study, the SPARCC scoring method, which shows the severity of sacroiliac joint inflammation, correlates with the clinical diagnosis of sacroiliitis. In cases with suspected sacroiliitis, except for extraordinary reasons, it can be evaluated with MRI without contrast material and can be graded to guide the clinician in treatment and approach.

Using Antinuclear Antibody Testing in Pediatric Rheumatology.

The antinuclear antibody (ANA) test is frequently used for the identification of patients who are at a high risk of developing autoimmune rheumatological diseases. The aim of this study is to evaluate the final diagnoses of patients applied to the pediatric rheumatology outpatient clinic with a positive ANA test result. In this study, the medical records of 283 children who had ANA positivity between January 2010 and January 2022 were evaluated retrospectively. All patients were younger than 18 years of age at diagnosis and were followed up in the pediatric rheumatology department for at least 6 months. The majority of the patients were females (69%), and the mean age was 9.9 ± 4.7 years. 94% of the ANA tests were requested in pediatric rheumatology outpatient clinics, and 6% in general pediatrics and other outpatient clinics. Arthritis was the most common reason for ANA testing (41.7%). Of the patients who had ANA positivity, 37% were diagnosed with juvenile idiopathic arthritis (JIA), 15% with connective tissue diseases, 10% with autoinflammatory disease, and 7% with vasculitides. Positivity at 1/320 and 1/640 titers were more common in the patients diagnosed with autoimmune connective tissue diseases or JIA compared to the patients without these diagnoses (P = .009 and P = .013, respectively). The ANA test should be judiciously requested by pediatric rheumatologists, especially in suspected cases of autoimmune rheumatic disorders and JIA patients to aid in classification. Indiscriminate use of the ANA test for screening may potentially misguide clinicians.

Efficacy and safety of an early response-based tapering regimen of tocilizumab in children with systemic juvenile idiopathic arthritis.

INTRODUCTION: Systemic juvenile idiopathic arthritis (sJIA) is a distinct disease subset, with a poorer prognosis compared with other JIA subsets. Tocilizumab has an important role in the management of sJIA refractory to standard initial therapy. However, no specific guidelines exist for the tapering of tocilizumab therapy in sJIA, which could have implications on the overall cost and side effects of treatment. METHODS: This was an observational study which included 21 children with refractory sJIA, who were initially put on injection tocilizumab every 2 weekly, with subsequent dosing tapered to 4 weekly and 6 weekly intervals based on JIA ACR 70 responses at 12 and 24 weeks, respectively. The primary outcome at week 36 included JIA ACR 30, 50, 70, and 90 response rates with other efficacy and safety measures as secondary outcomes. RESULTS: At 36 weeks, JIA ACR 30, 50, 70, and 90 responses were observed in 90.5%, 90.5%, 71.4%, and 52.4% patients respectively along with significant improvement in hematological and inflammatory parameters. The mean prednisolone dose could be reduced from 0.54 to 0.13 mg/kg/day and around 29% patients were able to discontinue steroids altogether. No serious adverse events were recorded. With drug tapering, we could curtail on 26% of the total tocilizumab dose that would have been otherwise required on the continuous 2 weekly protocol. CONCLUSIONS: Tocilizumab, used in an early response-based tapering regimen, was both safe and efficacious in children with sJIA refractory to standard therapy. Larger and longer duration studies are required to further validate our observations.