Development of a 3D cephalometric index to grade severity of dentofacial deformity in patients with juvenile idiopathic arthritis.

This investigation aimed to develop a radiographic 3D cephalometric index to grade severity of dentofacial deformity in patients with juvenile idiopathic arthritis (JIA), and to perform a validation against expert evaluations. Data were collected from a population-based Nordic JIA cohort of 240 patients that received a cone-beam computed tomography (CBCT) scan approximately 17 years after onset of JIA. The cohort was randomized into two groups: A baseline group for establishing the index (n = 210) and a test group (n = 30).

Effects of Biologics on Temporomandibular Joint Inflammation in Juvenile Idiopathic Arthritis.

Objective: This prospective study investigates the efficacy of biologics in combination with methotrexate (MTX) or leflunomide (LEF) on juvenile idiopathic arthritis (JIA)-related temporomandibular joint (TMJ) arthritis measured by magnetic resonance imaging (MRI)-based inflammation score and deformity score.

Methods: A prospective, single-center observational cohort study of 18 consecutive patients was performed between September 2018 and April 2023. Inclusion criteria were (1) diagnosis of JIA, (2) MRI-verified TMJ arthritis leading to treatment with tumor necrosis factor inhibitor (TNFi), (3) MRI at 6 months and 24 months after treatment initiation, and (4) clinical follow-up together with an MRI by a pediatric rheumatologist and an orthodontist.

Monozygotic triplets with juvenile-onset autoimmunity and 18p microdeletion involving .

Abnormal gene dosage from copy number variants has been associated with susceptibility to autoimmune disease. This includes 18p deletion syndrome, a chromosomal disorder with an estimated prevalence of 1 in 50,000 characterized by intellectual disability, facial dysmorphology, and brain abnormalities. The underlying causes for autoimmune manifestations associated with 18p deletions, however, remain unknown.

Inflammatory biomarkers predicting long-term remission and active disease in juvenile idiopathic arthritis: a population-based study of the Nordic JIA cohort.

Objectives: To assess the ability of baseline serum biomarkers to predict disease activity and remission status in juvenile idiopathic arthritis (JIA) at 18-year follow-up (FU) in a population-based setting.

Methods: Clinical data and serum levels of inflammatory biomarkers were assessed in the longitudinal population-based Nordic JIA cohort study at baseline and at 18-year FU. A panel of 16 inflammatory biomarkers was determined by multiplexed bead array assay.

Methotrexate Intolerance in Juvenile Idiopathic Arthritis: Definition, Risks, and Management.

Juvenile idiopathic arthritis is the most common rheumatic disorder in childhood and adolescence posing a significant threat of short-term and long-term disability if left untreated. Methotrexate is a folic acid analog with various immunomodulatory properties. It has demonstrated significant efficacy for the treatment of juvenile idiopathic arthritis, often considered the preferred first-line disease-modifying anti-rheumatic drug given as monotherapy or in combination with biological drugs.

Musculoskeletal misdiagnoses in pediatric patients with spinal tumors.

Objective: Childhood spinal tumors often present with musculoskeletal symptoms, potentially causing a misdiagnosis and delays in diagnosis and treatment. This study aims to identify, characterize, and compare children with spinal tumors who had prior musculoskeletal misdiagnoses to those without, analyzing clinical presentation, diagnostic interval, and outcome.

Study Design: This retrospective cohort study evaluated all children aged 0-14 years diagnosed with a spinal tumor in Denmark from 1996 to 2018.

Disease activity trajectories from childhood to adulthood in the population-based Nordic juvenile idiopathic arthritis cohort.

Objectives: To identify long-term disease activity trajectories from childhood to adulthood by using the clinical Juvenile Arthritis Disease Activity Score (cJADAS10) in juvenile idiopathic arthritis (JIA). Second, to evaluate the contribution of the cJADAS10 components and explore characteristics associated with active disease at the 18-year follow-up.

Methods: Patients with onset of JIA in 1997-2000 were followed for 18 years in the population-based Nordic JIA cohort.

Body mass index is associated with health-related quality of life and disease characteristics in young adults with juvenile idiopathic arthritis.

Background: There is a growing interest concerning the relationship between obesity and several medical conditions and inflammation. Nevertheless, there is a lack of studies regarding body mass index (BMI) among patients with juvenile idiopathic arthritis (JIA). Our aim was to investigate the impact of BMI on health-related quality of life (HRQoL) measured with a 36-Item Short Form Survey (SF-36), disease activity, and disability in young adults with JIA.

A novel serum calprotectin (MRP8/14) particle-enhanced immuno-turbidimetric assay (sCAL turbo) helps to differentiate systemic juvenile idiopathic arthritis from other diseases in routine clinical laboratory settings.

Background: Differential diagnosis in children with signs of unprovoked inflammation can be challenging. In particular, differentiating systemic juvenile idiopathic arthritis (SJIA) from other diagnoses is difficult. We have recently validated the complex of myeloid-related proteins 8/14 (MRP8/14, also known as S100A8/A9 complex or serum calprotectin) as a helpful biomarker supporting the diagnosis of SJIA.

Impact of physiotherapy on orofacial manifestations of juvenile idiopathic arthritis.

Background: Physiotherapy appears as a promising therapy option for patients with Juvenile Idiopathic Arthritis (JIA) [1, 2], but the effects of physiotherapy and jaw exercises on JIA-related orofacial symptoms remain unknown [3]. The aim of this proof-of-concept study was to assess the impact of orofacial physiotherapy and home-exercise programs in patients with JIA and temporomandibular joint (TMJ) involvement.

Methods: Twelve patients with JIA and TMJ involvement received a treatment of physiotherapy, complemented by prescribed home exercises spanning over eight weeks.

Biologic switching patterns among children with non-systemic juvenile idiopathic arthritis.

Background: In juvenile idiopathic arthritis (JIA) clinical remission is unattainable in some patients despite modern biologic disease-modifying antirheumatic drugs (bDMARD) therapy and switching bDMARD is required. The best choice of second-line bDMARD remains unclear. This retrospective observational study aims to describe the pattern, timing, frequency, and reasons for bDMARD switching among children diagnosed with non-systemic JIA.

Quantification of temporomandibular joint space in patients with juvenile idiopathic arthritis assessed by cone beam computerized tomography.

Objective: To describe a method to calculate the total intra-articular volume (inter-osseous space) of the temporomandibular joint (TMJ) determined by cone-beam computed tomography (CBCT). This could be used as a marker of tissue proliferation and different degrees of soft tissue hyperplasia in juvenile idiopathic arthritis (JIA) patients.

Materials And Methods: Axial single-slice CBCT images of cross-sections of the TMJs of 11 JIA patients and 11 controls were employed.

Musculoskeletal misdiagnoses in children with brain tumors: A nationwide, multicenter case-control study.

Objective: Childhood brain tumors belong to the cancer type with the longest diagnostic delay, the highest health care utilization prior to diagnosis, and the highest burden of long-term sequelae. We aimed to clarify whether prior musculoskeletal diagnoses in childhood brain cancer were misdiagnoses and whether it affected the diagnostic delay.

Study Design: In this retrospective, chart-reviewed case-control study we compared 28 children with brain tumors and a prior musculoskeletal diagnosis to a sex and age-matched control group of 56 children with brain tumors and no prior musculoskeletal diagnosis.

Changes in Kawasaki disease incidence and phenotype during the COVID-19 pandemic.

Introduction: The aetiology of Kawasaki disease (KD) remains unknown. Changes in infectious exposure during the COVID-19 pandemic owing to infection prevention measures may have affected the incidence of KD, supporting the pathogenic role of an infectious trigger. The purpose of this study was to evaluate the incidence, phenotype and outcome of KD before and during the COVID-19 pandemic in Denmark.

Phagocyte-related S100 proteins and cytokines in acute lymphoblastic leukemia and their prognostic value.

In this retrospective cohort study, we evaluated the level of biomarkers of inflammation like phagocyte-related S100 proteins and a panel of cytokines in 128 children with pre-B ALL and 22 with T-ALL. The biomarkers were evaluated at diagnosis and during antileukemic therapy (day 29 and after six months) and we evaluated their correlation with basic laboratory values. Further, for the children with pre-B ALL, we evaluated whether the biomarkers could predict the outcome of ALL expressed as minimal residual disease (MRD), relapse, and death.

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.

Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles.

Objectives: The 2016 ACR-EULAR Response Criteria for JDM was developed as a composite measure with differential weights of six core set measures (CSMs) to calculate a Total Improvement Score (TIS). We assessed the contribution of each CSM, representation of muscle-related and patient-reported CSMs towards improvement, and frequency of CSM worsening across myositis response criteria (MRC) categories in validation of MRC.

Methods: Data from JDM patients in the Rituximab in Myositis trial (n = 48), PRINTO JDM trial (n = 139), and consensus patient profiles (n = 273) were included.

The role of liver transaminase levels in methotrexate intolerance in juvenile idiopathic arthritis-a cross-sectional study.

Methotrexate (MTX) plays a key role when treating juvenile idiopathic arthritis (JIA), but MTX-intolerance is challenging. MTX-treatment might affect the liver, causing elevated levels of alanine aminotransferase (ALT), yet the role of ALT-levels in MTX-intolerance in JIA remains unclear. Our study aimed to investigate the association between ALT-levels during MTX-treatment and MTX-intolerance in JIA.

Incidence of Orofacial Manifestations of Juvenile Idiopathic Arthritis From Diagnosis to Adult Care Transition: A Population-Based Cohort Study.

Objective: To estimate the cumulative incidences of orofacial conditions related to temporomandibular joint (TMJ) juvenile idiopathic arthritis (JIA) between diagnosis in childhood to transition into adult care, and to identify features in JIA associated with TMJ involvement.

Methods: A population-based cohort analysis was conducted of patients with JIA involving longitudinal data on orofacial health from 2000 to 2018. Regardless of TMJ status, the patients were referred to the Regional Specialist Craniofacial Clinic of Western Denmark for routine orofacial examinations.

Assessment of Orofacial Symptoms in Juvenile Idiopathic Arthritis: Validation of a Consensus-Based Short Patient Questionnaire.

Objective: To develop, validate, and test the performance of patient-reported outcomes (PROs) in a short patient questionnaire (< 5 minutes in length) in order to assess the multidimensional aspects of orofacial symptoms related to juvenile idiopathic arthritis (JIA) among patients aged ≥ 10 years.

Methods: The study was conducted by an interdisciplinary task force from the Temporomandibular Joint Juvenile Arthritis Working Group. The project consisted of a multiphased approach including the following: (1) conceptual phase with online international survey of 167 healthcare workers, (2) item generation and drafting of preliminary questionnaire(s) (ie, face validity), (3) cognitive script interview and probing (ie, content validity; 16 patients with JIA), (4) assessment of construct validity (ie, convergence and divergence; 53 patients with JIA), (5) test of reliability, and (6) test of clinical performance and psychometric characteristics (95 patients with JIA).

Standardized reporting and quantification of whole-body MRI findings in children with chronic non-bacterial osteomyelitis treated with pamidronate.

Objectives: The objectives were to assess changes in radiological disease activity in children with chronic non-bacterial osteomyelitis (CNO) receiving pamidronate therapy and to test a modified radiological index for non-bacterial osteitis (mRINBO) in CNO. mRINBO was used for standardized reporting and quantification of whole-body MRI (WBMRI) findings resulting in an individual summary patient score.

Methods: WBMRI was retrospectively assessed in 18 children with CNO at baseline and after receiving pamidronate therapy for one year.

Management of Orofacial Manifestations of Juvenile Idiopathic Arthritis: Interdisciplinary Consensus-Based Recommendations.

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration.