Transcriptomic signatures of classical monocytes reveal pro-inflammatory modules and heterogeneity in polyarticular juvenile idiopathic arthritis.

Introduction: Polyarticular juvenile idiopathic arthritis (pJIA) is a childhood-onset autoimmune disease. Immune cells contribute to persistent inflammation observed in pJIA. Despite the crucial role of monocytes in arthritis, the precise involvement of classical monocytes in the pathogenesis of pJIA remains uncertain. Here, we aimed to uncover the transcriptomic patterns of classical monocytes in pJIA, focusing on their involvement in disease mechanism and heterogeneity. Methods: A total of 17 healthy subjects and 18 premenopausal women with pJIA according to ILAR criteria were included. Classical monocytes were isolated, and RNA sequencing was performed. Differential expression analysis was used to compare pJIA patients and healthy control group. Differentially expressed genes (DEGs) were identified, and gene set enrichment analysis (GSEA) was performed. Using unsupervised learning approach, patients were clustered in two groups based on their similarities at transcriptomic level. Subsequently, these clusters underwent a comparative analysis to reveal differences at the transcriptomic level. Results: We identified 440 DEGs in pJIA patients of which 360 were upregulated and 80 downregulated. GSEA highlighted TNF-α and IFN-γ response. Importantly, this analysis not only detected genes targeted by pJIA therapy but also identified new modulators of immuno-inflammation. PLAUR, IL1B, IL6, CDKN1A, PIM1, and ICAM1 were pointed as drivers of chronic hyperinflammation. Unsupervised learning approach revealed two clusters within pJIA, each exhibiting varying inflammation levels. Conclusion: These findings indicate the pivotal role of immuno-inflammation driven by classical monocytes in pJIA and reveals the existence of two subclusters within pJIA, regardless the positivity of rheumatoid factor and anti-CCP, paving the way to precision medicine.

Presentation of enthesitis-related arthritis and juvenile-onset spondyloarthritis: a cross-sectional study in a pediatric and adult clinic.

BACKGROUND: Juvenile idiopathic arthritis (JIA) comprises a whole spectrum of chronic arthritis starting before 16 years of age. The study aims to explore the clinical and demographic descriptors, treatment, and disease progression of enthesitis-related arthritis (ERA) in comparison with juvenile-onset spondyloarthritis (SpA). METHODS: Cross-sectional analysis of consecutive patients in two dedicated clinics, with a single visit and retrospective case-notes review. Arthritis, enthesitis and sacroiliitis were evaluated by scoring disease activity and damage. Continuous variables were reported by median, interquartile range; categorical variables were reported by the frequency comparison of the two groups. RESULTS: Thirty-three cases were included, being 23 (69.7%) with ERA. The median age at diagnosis was 12.5 y (SpA) vs. 9 y (ERA) (p < 0.01); the time from symptom onset to diagnosis was 5.5 y (SpA) vs. 1.5 y (ERA) (p < 0.03). In both groups, the predominant presentation was a single joint or < 5 lower limb joints and asymmetric involvement, with a high frequency of enthesitis. There was a higher frequency of mid-tarsal and ankle synovitis in the ERA group and hip involvement in those with SpA. The comparison of the frequency of spine symptoms at presentation, 30% SpA vs. 21.7% ERA (p = 0.7), was not significant, and radiographic progression to spinal involvement occurred in 43.5% of ERA patients. The median time for spinal progression and age at onset was 2.2 and 12 y for ERA, and 4 and 16.5 y for SpA, respectively. Activity and damage scores were not significantly different between the groups. Treatment comparison resulted in 91.3% of ERA and 100% SpA being treated, predominantly with NSAIDs in both groups, followed by DMARDs and biologics, with a higher frequency of biologics in SpA. CONCLUSION: The main differences were the late diagnoses of SpA, and the hip and spine involvement, with higher frequency of biologic treatment in juvenile-onset SpA compared to ERA.

Rhupus syndrome in the pediatric population: A comprehensive systematic literature review.

INTRODUCTION: The term "Rhupus" was employed to descriptively illustrate the overlap observed in some pediatric patients displaying features of both juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE). Although "Rhupus" is traditionally used in adults, we applied it broadly to emphasize this clinical overlap. METHODS: We sought to identify studies that registered signs, symptoms, imaging characteristics, and treatments given to patients with JIA and SLE. We searched four databases using a Boolean search string, resulting in 231 articles after duplicate removal. Title and abstract screening yielded 57 articles for full-text assessment. Full reviewed 13 extracted data regarding sex, age of onset, serologic and imaging findings, and management strategies. The NIH quality assessment tool was applied to ensure the internal validity of the articles. RESULTS: From the 13 articles evaluated that meet inclusion criteria, none had standardized diagnostic algorithms. The total number of patients in those articles is 26, without discussing treatment guidelines. DISCUSSION: Clinical presentation, diagnostic parameters, and treatment of pediatric Rhupus were synthesized in this review. Fundamental keys help distinguish the joint presentation when Juvenile Idiopathic Arthritis or Lupus is present, compared with the signs and symptoms when developing the overlapping syndrome. We highlight the importance of physicians knowing about this rare condition and call all specialists to report new cases of the disease so a consensus can be reached to establish standardized guidelines for diagnosing and treating Rhupus syndrome.

Health Care Utilization and Direct Costs Prior to Subspecialty Care in Children with Chronic Pain Compared with Other Chronic Childhood Diseases: A Cohort Study.

OBJECTIVES: To understand the burden associated with pediatric chronic pain (CP) on the health care system compared with other costly chronic diseases prior to subspecialty care. STUDY DESIGN: In this retrospective cohort study, we assessed all-cause health care utilization and direct health care costs associated with pediatric CP (n = 91) compared with juvenile arthritis (n = 135), inflammatory bowel disease (n = 90), type 1 diabetes (n = 475) or type 2 diabetes (n = 289), anxiety (n = 7193), and controls (n = 273) 2 and 5 years prior to patients entering subspecialty care in Manitoba, Canada. Linked data from physician encounters, emergency department visits, hospitalizations, and prescriptions were extracted from administrative databases. Differences in health care utilization and direct health care costs associated with CP vs the other conditions were tested using negative binomial and zero-inflated negative binomial regression models, respectively. RESULTS: After adjustment for age at diagnosis, sex, location of residence, and socioeconomic status, CP continued to be associated with the highest number of consulted physicians and subspecialists and the highest number of physician billings compared with all other conditions (P < .01, respectively). CP was significantly associated with higher physician costs than juvenile arthritis, inflammatory bowel disease, type 1 diabetes, type 2 diabetes, or controls (P < .01, respectively); anxiety was associated with the highest physician and prescription costs among all cohorts (P < .01, respectively). CONCLUSION: Compared with chronic inflammatory and endocrinologic conditions, pediatric CP and anxiety were associated with substantial burden on the health care system prior to subspecialty care, suggesting a need to assess gaps and resources in the management of CP and mental health conditions in the primary care setting.

Total hip arthroplasty in adolescents: a systematic review and meta-analysis.

PURPOSE: Total hip arthroplasty (THA) has demonstrated excellent results in elderly patients, however, the indications, outcomes, and long-term results in adolescent patients are less understood. This study aims to assess the outcomes of THA in patients under 21, providing insights for clinical decision-making in this exceptional population. METHODS: A systematic review in PubMed, Ovid MEDLINE, and Embase database was performed. We included studies reporting clinical, radiological, and functional outcomes of THA in patients younger than 21 years, for any cause, with a with a minimum follow-up of one year. The ten year survivorship estimate was pooled using a meta-analysis methodology and each study was weighted according to its standard error, calculated from published confidence intervals. RESULTS: We included 25 studies involving 1166 hips. Median age was 17 years old, 60% were females, and the average follow-up was 8.1 years. Juvenile inflammatory arthritis was the main indication for total hip arthroplasty (THA). The all-cause revision rate was 14.4% and aseptic loosening was the most common cause. Only eight studies reported ten year survival rates and form the pooled analysis an 84.91% survival rate (95% CI 70.56 - 99.27) was obtained. An average score of 88.08 in the Harris Hip Score (HHS) was observed. We found a 3.43% complication rate. CONCLUSIONS: Hip arthroplasty is an acceptable option for adolescents with end-stage arthritis. However, the altered hip anatomy, the elevated revision rate, and the long-term implant survival must be considered before performing a THA in adolescent patients.

Genome Sequencing in an Individual Presenting with 22q11.2 Deletion Syndrome and Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis is a heterogeneous group of diseases characterized by arthritis with poorly known causes, including monogenic disorders and multifactorial etiology. 22q11.2 proximal deletion syndrome is a multisystemic disease with over 180 manifestations already described. In this report, the authors describe a patient presenting with a short stature, neurodevelopmental delay, and dysmorphisms, who had an episode of polyarticular arthritis at the age of three years and eight months, resulting in severe joint limitations, and was later diagnosed with 22q11.2 deletion syndrome. Investigation through Whole Genome Sequencing revealed that he had no pathogenic or likely-pathogenic variants in both alleles of the MIF gene or in genes associated with monogenic arthritis (LACC1, LPIN2, MAFB, NFIL3, NOD2, PRG4, PRF1, STX11, TNFAIP3, TRHR, UNC13DI). However, the patient presented 41 risk polymorphisms for juvenile idiopathic arthritis. Thus, in the present case, arthritis seems coincidental to 22q11.2 deletion syndrome, probably caused by a multifactorial etiology. The association of the MIF gene in individuals previously described with juvenile idiopathic arthritis and 22q11.2 deletion seems unlikely since it is located in the distal and less-frequently deleted region of 22q11.2 deletion syndrome.

Evaluation of the safety profile of the quadrivalent vaccine against human papillomavirus in the risk of developing autoimmune, neurological, and hematological diseases in adolescent women in Colombia.

BACKGROUND: Cervical cancer eradication is one of the main goals for 2030 by the World Health Organization, which can only be achieved with high vaccination rates against Human Papilloma Virus. In Colombia, more and better scientific evidence is required to increase confidence in vaccination. The objective of this study is to evaluate the safety profile of the quadrivalent vaccine against HPV in the risk of developing autoimmune, neurological, and hematological diseases in adolescent women in Colombia. METHODS: We designed a cohort study based on national HPV vaccination records and incident diagnostic data for the diseases of special interest during 2012 and 2021. We included adolescent women between 9 and 19 years old and compared vaccinated and non-vaccinated cohorts using an Inverse Probability of Treatment Weighting (IPWT) method for each scenario disease and follow-up period (180 and 360 days). FINDINGS: The Odds Ratio (OR) of developing diseases of interest was estimated during two follow up periods, 180 and 360 days after the follow-up index date (Vaccination Day). The OR for developing rheumatoid arthritis was 4·4; CI95% (1·74 - 11·14), juvenile idiopathic arthritis was 2·76 IC95% (1·50 - 5·11), idiopathic thrombocytopenic purpura was 2·54 IC95% (1·28 - 5·02) and thyrotoxicosis was 2·86 IC95% (1·03 - 7·95), when comparing the vaccinated versus unvaccinated population. However, the temporal distribution of cases incident did not reveal a clear difference between the cohorts, since the rate of appearance of new cases has a constant linear behavior for the two groups. INTERPRETATION: For rheumatoid arthritis, juvenile idiopathic arthritis, idiopathic thrombocytopenic purpura, and thyrotoxicosis; the application of the vaccine had an effect on the development of the disease. Nevertheless, our results should be interpreted with caution and be further studied, considering that the biological plausibility of the events occurred without a clear temporal pattern in relation to the exposure to the vaccine.

Low prevalence of subclinical synovitis in patients with juvenile idiopathic arthritis (JIA) in long-term clinical remission on medication.

Subclinical synovitis is highly prevalent in patients with JIA in clinical remission (CR) with a short duration. The objective was to evaluate its prevalence by ultrasound (US) in patients with JIA in long CR during a one-year follow-up. In this prospective and longitudinal study, we included 76 patients with JIA according to ILAR with CR by the Wallace modified criteria and JADAS27 and compared them with 22 patients with active disease. Clinical and demographic characteristics were recorded. US evaluation was by 10-joint count. Differences in US evaluations were analyzed by the Mann-Whitney U test. There were no differences among the two group with regard to disease duration at enrollment, and age (p = 0.540 and p = 0.080, respectively), but JADAS 27, CHAQ, and acute phase reactants were significantly higher (p < 0.001) in the clinically active group. The prevalence of subclinical synovitis at baseline and the end of the study in the CR group was 18.4% and 11.8%, respectively, while it was 100% and 40.9% in the active disease group. Subclinical synovitis at baseline was significantly more prevalent in the clinically active group (elbow, p = 0.01; wrist, p = 0.001; MCP 2, p = 0.001; knee, p = 0.001 and ankle p = 0.001; and PD only in the ankle, p = 0.002). The concordance of inter-reader reliability in all evaluated joints was excellent (p = 0.001). Although the prevalence of subclinical synovitis is low in patients with JIA with long-term clinical remission on medication, a percentage of patients continue to have subclinical involvement that could predict the risk of relapse and structural damage. Key Points • Subclinical synovitis is less prevalent in JIA in long-term clinical remission compared to patients in short-term remission. • The persistence of imaging signs of inflammation in a significant percentage of patients may indicate the need for ongoing medication.

Bone erosions associated with systemic bone loss on HR-pQCT in women with longstanding polyarticular juvenile idiopathic arthritis.

OBJECTIVES: To analyze longstanding polyarticular juvenile idiopathic arthritis (pJIA) for possible associations between localized bone damage (erosions), and systemic bone loss. Besides, to compare the systemic bone mass of pJIA with healthy controls. METHODS: Thirty-four pJIA women and 99 healthy controls (HC) were included. Radius and tibia of all subjects were scanned by HR-pQCT. Volumetric bone mineral density (vBMD), bone microarchitecture, and -finite element parameters were analyzed. Patients underwent HR-pQCT of 2nd and 3rd metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints of the dominant hand, for bone erosions quantification. RESULTS: The mean age of patients was 31.5 ± 7.4yrs with a mean disease duration of 21.7 ± 9.2yrs. Bone erosions were detectable in 79% of patients. The number of bone erosions was positively correlated with cortical porosity (Ct.Po) at tibia (r = 0.575, p = 0.001), and radius (r = 0.423, p = 0.018); and negatively correlated with cortical vBMD at tibia (r=-0.420, p = 0.015). In a logistic regression analysis, adjusted for anti-CCP, the presence of bone erosions was independently associated with Ct.Po at radius (p = 0.018) and cortical vBMD at tibia (p = 0.020). Moreover, cortical and trabecular vBMD, trabecular number, and µ-finite element parameters were decreased in patients compared to HC (p < 0.05). CONCLUSION: Bone erosions in longstanding pJIA women were associated with decreased cortical bone parameters, and these patients showed systemic bone impairment at peripheral sites compared with healthy controls.

Physical activity and education about physical activity for chronic musculoskeletal pain in children and adolescents.

BACKGROUND: Chronic pain is a major health and socioeconomic burden, which is prevalent in children and adolescents. Among the most widely used interventions in children and adolescents are physical activity (including exercises) and education about physical activity. OBJECTIVES: To evaluate the effectiveness of physical activity, education about physical activity, or both, compared with usual care (including waiting-list, and minimal interventions, such as advice, relaxation classes, or social group meetings) or active medical care in children and adolescents with chronic musculoskeletal pain. SEARCH METHODS: We searched CENTRAL, MEDLINE, Embase, CINAHL, PsycINFO, PEDro, and LILACS from the date of their inception to October 2022. We also searched the reference lists of eligible papers, ClinicalTrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Platform. SELECTION CRITERIA: We included randomised controlled trials (RCTs) that compared physical activity or education about physical activity, or both, with usual care (including waiting-list and minimal interventions) or active medical care, in children and adolescents with chronic musculoskeletal pain. DATA COLLECTION AND ANALYSIS: Two review authors independently determined the eligibility of the included studies. Our primary outcomes were pain intensity, disability, and adverse events. Our secondary outcomes were depression, anxiety, fear avoidance, quality of life, physical activity level, and caregiver distress. We extracted data at postintervention assessment, and long-term follow-up. Two review authors independently assessed risk of bias for each study, using the RoB 1. We assessed the overall certainty of the evidence using the GRADE approach. We reported continuous outcomes as mean differences, and determined clinically important differences from the literature, or 10% of the scale. MAIN RESULTS: We included four studies (243 participants with juvenile idiopathic arthritis). We judged all included studies to be at unclear risk of selection bias, performance bias, and detection bias, and at high risk of attrition bias. We downgraded the certainty of the evidence for each outcome to very low due to serious or very serious study limitations, inconsistency, and imprecision. Physical activity compared with usual care Physical activity may slightly reduce pain intensity (0 to 100 scale; 0 = no pain) compared with usual care at postintervention (standardised mean difference (SMD) -0.45, 95% confidence interval (CI) -0.82 to -0.08; 2 studies, 118 participants; recalculated as a mean difference (MD) -12.19, 95% CI -21.99 to -2.38; I² = 0%; very low-certainty evidence). Physical activity may slightly improve disability (0 to 3 scale; 0 = no disability) compared with usual care at postintervention assessment (MD -0.37, 95% CI -0.56 to -0.19; I² = 0%; 3 studies, 170 participants; very low-certainty evidence). We found no clear evidence of a difference in quality of life (QoL; 0 to 100 scale; lower scores = better QoL) between physical activity and usual care at postintervention assessment (SMD -0.46, 95% CI -1.27 to 0.35; 4 studies, 201 participants; very low-certainty evidence; recalculated as MD -6.30, 95% CI -18.23 to 5.64; I² = 91%). None of the included studies measured adverse events, depression, or anxiety for this comparison. Physical activity compared with active medical care We found no studies that could be analysed in this comparison. Education about physical activity compared with usual care or active medical care We found no studies that could be analysed in this comparison. Physical activity and education about physical activity compared with usual care or active medical care We found no studies that could be analysed in this comparison. AUTHORS' CONCLUSIONS: We are unable to confidently state whether interventions based on physical activity and education about physical activity are more effective than usual care for children and adolescents with chronic musculoskeletal pain. We found very low-certainty evidence that physical activity may reduce pain intensity and improve disability postintervention compared with usual care, for children and adolescents with juvenile idiopathic arthritis. We did not find any studies reporting educational interventions; it remains unknown how these interventions influence the outcomes in children and adolescents with chronic musculoskeletal pain. Treatment decisions should consider the current best evidence, the professional's experience, and the young person's preferences. Further randomised controlled trials in other common chronic musculoskeletal pain conditions, with high methodological quality, large sample size, and long-term follow-up are urgently needed.

Inflammatory Biomarkers Can Differentiate Acute Lymphoblastic Leukemia with Arthropathy from Juvenile Idiopathic Arthritis Better Than Standard Blood Tests.

OBJECTIVE: To evaluate the predictive value of biomarkers of inflammation like phagocyte-related S100 proteins and a panel of inflammatory cytokines in order to differentiate the child with acute lymphoblastic leukemia (ALL) from juvenile idiopathic arthritis (JIA). STUDY DESIGN: In this cross-sectional study, we measured S100A9, S100A12, and 14 cytokines in serum from children with ALL (n = 150, including 27 with arthropathy) and JIA (n = 236). We constructed predictive models computing areas under the curve (AUC) as well as predicted probabilities in order to differentiate ALL from JIA. Logistic regression was used for predictions of ALL risk, considering the markers as the respective exposures. We performed internal validation using repeated 10-fold cross-validation and recalibration, adjusted for age. RESULTS: In ALL, the levels of S100A9, S100A12, interleukin (IL)-1 beta, IL-4, IL-13, IL-17, matrix metalloproteinase-3, and myeloperoxidase were low compared with JIA (P < .001). IL-13 had an AUC of 100% (95% CI 100%-100%) due to no overlap between the serum levels in the 2 groups. Further, IL-4 and S100A9 had high predictive performance with AUCs of 99% (95% CI 97%-100%) and 98% (95% CI 94%-99%), respectively, exceeding both hemoglobin, platelets, C-reactive protein, and erythrocyte sedimentation rate. CONCLUSIONS: The biomarkers S100A9, IL-4, and IL-13 might be valuable markers to differentiate ALL from JIA.

Does the use of panoramic radiography add information in the temporomandibular joint evaluation in Juvenile Idiopathic Arthritis patients? A case control study.

OBJECTIVE: To determine the frequency of radiographic changes in the temporomandibular joint, in a representative population of patients with Juvenile Idiopathic Arthritis (JIA) and to compare with findings in healthy controls matched by sex and age. PATIENTS AND METHODS: One hundred and thirty-seven panoramic radiographies (PR) from JIA patients of a pediatric rheumatology outpatient clinic were prospectively evaluated and compared to 137 PR from healthy individuals. RESULTS: 102 (74.5%) JIA patients and 47 (34.3%) controls showed at least one radiological alteration (p < 0.001). The following radiographic alterations were more frequently observed in JIA patients than in controls: erosion (p < 0.001), altered condylar morphology (p < 0.001), disproportion between condylar process and the coronoid process (p < 0.001) and accentuated curve in the antegonial notch (p = 0.002). Twenty patients (14.6%) presented the four radiographic alterations simultaneously compared to only two controls (1.5%) (p < 0.001). CONCLUSION: Due to the difference in the frequency of findings in the PR of patients and controls, we concluded that PR has value as a screening tool. In the presence of major changes in the mandible head in the PR of patients with a confirmed diagnosis of JIA, MRI should be considered to detect an active inflammatory process in this joint.

Therapeutic plasma exchange in refractory macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a case-based review.

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of arthritis of autoimmune aetiology. Systemic-onset juvenile idiopathic arthritis (soJIA) presents with fever, transient erythematous rash, hepatomegaly, splenomegaly, lymphadenopathy, and serositis. SoJIA presents multiple complications, and the most severe is the macrophage activation syndrome (MAS); the timely treatment of MAS must be established early and aggressively to avoid a fatal outcome. Therapeutic plasma exchange has anecdotally been used in refractory cases. A 66-month-old male with a 1-year illness characterized by evening-predominant, intermittent fever, adenomegalies, urticarial-like rash, arthralgia, and arthritis. Biochemical analysis revealed anaemia, leukocytosis, neutrophilia, hypertriglyceridemia, hyperferritinemia, and hypofibrinogenemia; bone marrow aspirate showed hemophagocytosis. He was diagnosed with SoJIA complicated with MAS. He received multiple treatments with IV human gammaglobulin, cyclosporine, dexamethasone, and tocilizumab without improvement. Plasma replacement treatment was performed. Afterwards, he presented significant improvement. After 3-year-follow-up, he remains in good general condition. We present a refractory case of soJIA complicated with MAS successfully treated with plasma exchange.

Development and Initial Validation of the ONCOREUM Score to Differentiate Childhood Cancer with Arthropathy from Juvenile Idiopathic Arthritis.

OBJECTIVE: To develop and validate a weighted score, the ONCOREUM score, that aids physicians in differentiation of cancer with arthropathy from juvenile idiopathic arthritis (JIA). STUDY DESIGN: Data were extracted from the ONCOREUM Study, a multicenter, cross-sectional investigation aimed at comparing children with cancer and arthropathy to children with JIA. Three statistical approaches were applied to develop the ONCOREUM score and assess the role of each variable in the diagnosis of cancer with arthropathy, including 2 approaches based on multivariable stepwise selection (models 1 and 2) and 1 approach on a Bayesian model averaging method (model 3). The ß coefficients estimated in the models were used to assign score points. Considering that not missing a child with cancer is a mandatory clinical objective, discriminating performance was assessed by fixing sensitivity at 100%. Score performance was evaluated in both developmental and validation samples (representing 80% and 20% of the study population, respectively). RESULTS: Patients with cancer and arthropathy (49 with solid tumors and 46 with hematologic malignancies without peripheral blasts) and 677 patients with JIA were included. The highest area under the receiver operating characteristic (ROC) curve (AUC) in the validation data set was yielded by model 1, which was selected to constitute the ONCOREUM score. The score ranged from -18 to 21.8, and the optimal cutoff obtained through ROC analysis was -6. The sensitivity, specificity, and AUC of the cutoff in the validation sample were 100%, 70%, and 0.85, respectively. CONCLUSIONS: The ONCOREUM score is a powerful and easily applicable tool that may facilitate early differentiation of malignancies with articular complaints from JIA.

What is the image appearance of juvenile idiopathic arthritis in MRI, CT, and CBCT of TMJ? A systematic review.

OBJECTIVE: We performed a systematic review to investigate the appearance of imaging signs on magnetic resonance imaging (MRI), cone-beam computed tomography (CBCT), and conventional computed tomography (CT) scans of the temporomandibular joints (TMJs) of patients with juvenile idiopathic arthritis (JIA). MATERIALS AND METHODS: We performed electronic searches of the PubMed, Embase, Web of Science, Scopus, Lilacs, and the Cochrane Library databases to identify studies investigating JIA and its related imaging findings. Inclusion criteria were as follows: original article studies based on humans and systematic reviews, studies enrolling patients under 18 years of age with a diagnostic of JIA, the use of International League of Associations for Rheumatology (ILAR) criteria and one type of medical imaging (MRI, CBCT, or CT), and papers published in the English language. RESULTS: A total of six studies met the inclusion criteria, four involving MRI and two involving CBCT. Additionally, all six studies analyzed the imaging findings of pathological TMJ affected by JIA. The results showed that synovial membrane enhancement, condylar erosions, and condylar flattening were the most prevalent imaging findings in JIA. CONCLUSION: MRI examinations are more specific for detecting anomalies in the TMJ than CBCT and CT. Additionally, these results must be correlated with clinical signs to verify the correct diagnosis. CLINICAL RELEVANCE: This study identified the most prevalent imaging signs of JIA to provide an early and correct diagnosis of the disease.

Diagnosis of juvenile idiopathic arthritis with asymptomatic involvement of the temporomandibular joint: A case report.

Juvenile idiopathic arthritis (JIA) is a broad term that describes a group of heterogeneous rheumatologic diseases, mainly characterized by inflammation in the joints of children and young people up to 16 years of age. Its etiology is still not well understood and the diagnosis, essentially clinical, begins with the exclusion of other joint diseases. When the disease affects the temporomandibular joint, diagnosis is a challenge, as many patients are asymptomatic. The objective of this paper is to present a case of JIA with severe involvement of the temporomandibular joint and to discuss the clinical, radiographic, laboratory findings and the importance of early diagnosis. The lack of diagnosis of active arthritis in the temporomandibular joint in patients with JIA can cause irreversible effects such as micrognathia, malocclusion and reduced maximum mouth opening. Early diagnosis of temporomandibular joint involvement in JIA is important and needs to be investigated early in the clinical manifestation of systemic disease. Laboratory tests and clinical history are important to define treatment and prognosis, but not to predict temporomandibular joint arthritis. Imaging exams are important diagnostic tools to identify morphological changes in soft and hard tissues of the temporomandibular joint.

Magnetic resonance imaging texture analysis for quantitative evaluation of the mandibular condyle in juvenile idiopathic arthritis.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease that affects the joints and other organs, including the development of the former in a growing child. This study aimed to evaluate the feasibility of texture analysis (TA) based on magnetic resonance imaging (MRI) to provide biomarkers that serve to identify patients likely to progress to temporomandibular joint damage by associating JIA with age, gender and disease onset age. METHODS: The radiological database was retrospectively reviewed. A total of 45 patients were first divided into control group (23) and JIA group (22). TA was performed using grey-level co-occurrence matrix (GLCM) parameters, in which 11 textural parameters were calculated using MaZda software. These 11 parameters were ranked based on the p value obtained with ANOVA and then correlated with age, gender and disease onset age. RESULTS: Significant differences in texture parameters of condyle were demonstrated between JIA group and control group (p < 0.05). There was a progressive loss of uniformity in the grayscale pixels of MRI with an increasing age in JIA group. CONCLUSIONS: MRI TA of the condyle can make it possible to detect the alterations in bone marrow of patients with JIA and promising tool which may help the image analysis.

Multiple Sclerosis in a Patient with Intermediate Uveitis and Juvenile Idiopathic Arthritis Treated with Adalimumab: A Case Report.

PURPOSE: To report a case of multiple sclerosis (MS) development in a patient with Juvenile Idiopathic Arthritis (JIA) and bilateral intermediate uveitis (IU) treated with Adalimumab. CASE REPORT: A 21-year-old Colombian woman diagnosed with JIA and bilateral refractory IU treated with methotrexate and Adalimumab with difficult control of the disease and multiple ocular complications. Eight years after starting Adalimumab, the patient presented paresthesia in the left upper limb. Radiologic findings in the brain and cervical spine MRI confirmed the diagnosis of MS. CONCLUSIONS: We reported the first case of MS development in a patient with JIA treated with Adalimumab and the third in a patient with noninfectious uveitis treated with anti-TNFα. It remains uncertain whether MS is secondary to anti-TNFα therapy or is linked to a polyautoimmunity phenomenon.

Optic neuromyelitis in relation with juvenile idiopathic arthritis: A case report

Optic neuromyelitis (ONM), also called neuromyelitis optica spectrum (Neuromyelitis Optica Spectrum Disorders, NMOSD) is recognized as an inflammatory autoimmune demyelinating disease of the central nervous system, mediated by autoantibodies against the aquaporin-4 receptor (AQP4-IgG). It predominantly affects the optic nerves and the spinal cord.1-3 It is known that patients with immune disorders are more likely to present other autoimmune diseases, but the relation between juvenile idiopathic arthritis and ONM has not been completely described.5 In this paper, we report a case of a patient with juvenile idiopathic arthritis, presenting with a rapidly progressive neurological condition, who is treated with biological drugs.1-4

La neuromielitis óptica (NMO), también llamada espectro de la neuromielitis óptica (neuromyelitis optica spectrum disorders) se reconoce como una enfermedad inflamatoria, autoinmune, desmielinizante del sistema nervioso central, mediada por autoanticuerpos contra el receptor de acuaporina 4 (AQP4-IgG) que afecta predominantemente a los nervios ópticos y la médula espinal1-3. Es conocido que los pacientes con trastornos inmunitarios tienen más probabilidades de presentar otras enfermedades autoinmunes; sin embargo, no está completamente descrita la asociación entre artritis idiopática juvenil y NMO5. En este escrito se reporta el caso de una paciente que cursa con artritis idiopática juvenil, inició con compromiso neurológico rápidamente progresivo, y es tratada con medicamentos biológicos1-4.