Factors associated with poor prognosis of hip arthritis in juvenile idiopathic arthritis: Data from the JIR cohort.

OBJECTIVES: Hip involvement remains a predictor of severe juvenile idiopathic arthritis (JIA) course and carries a high risk of disability. This study aims to determine the factors of poor prognosis of hip involvement in patients with JIA and to assess the treatment response. METHODS: This is a multicenter observational cohort study. Patients were selected from the JIR Cohort database. Hip involvement was defined as clinically suspected and confirmed by an imaging tool. Follow-up data were collected during 5 years. RESULTS: Among the 2223 patients with JIA, 341(15%) patients had hip arthritis. Male gender, enthesitis-related arthritis, and North African origin were factors associated with hip arthritis. Hip inflammation was associated with disease activity parameters during the first year, particularly Physician Global Assessment, joint count, and inflammatory marks. Structural hip progression was associated with early onset of the disease, a longer time to diagnosis, geographic origin, and JIA subtypes. Anti-TNF therapy was found to be the only treatment able to effectively reduce structural damage progression. CONCLUSION: The early onset diagnostic delay, origin, and systemic subtype of JIA predict a poor prognosis of hip arthritis in children with JIA. The use of anti-TNF was associated with a better structural prognosis.

Individual and environmental determinants associated with longer times to access pediatric rheumatology centers for patients with juvenile idiopathic arthritis, a JIR cohort study.

BACKGROUND: Despite guidelines, poor access to appropriate care for juvenile idiopathic arthritis (JIA) patients remains a global issue. Prompt referral to a pediatric rheumatology (PR) center and effective care is known to be critical for changing the natural history of the disease and improving long-term prognosis. This project assesses socio-economic factors of delayed referral to a pediatric rheumatologist (PRst) for JIA patients in France and Switzerland within the Juvenile Inflammatory Rheumatism (JIR) Cohort. METHODS: All patients diagnosed with JIA, presenting at one center of the JIRcohort in France or Switzerland with additional data on referral pathway were included. Patient characteristics at first visit to the PR center, dates of visits to healthcare providers during referral, and parent characteristics were extracted from the JIRcohort database. RESULTS: Two hundred fifty children were included. The overall median time to first PR assessment was 2.4 months [1.3; 6.9] and ranged widely across the JIA subtypes, from 1.4 months [0.6; 3.8] for children with systemic juvenile idiopathic arthritis (sJIA) to 5.3 months [2.0; 19.1] for children with enthesitis-related arthritis (ERA). A diagnosis of ERA and an appointment with an orthopedist during the referral pathway were significantly associated with a longer time before the first PR visit (hazard ratio HR 0.50 [95% CI: 0.29; 0.84]) and HR 0.68 [95% CI: 0.49; 0.93], respectively) in multivariable analysis. Having a mother with a post-graduate educational attainment level was tendentially associated with a shorter time before the first PR visit, (HR 1.32 [95% CI: 0.99; 1.78]). CONCLUSIONS: Time to first PRst visit was most often short compared to other studies and close to the British recommendations. However, this time remained too long for many patients. We observed no social inequities in access to a PRst, but we show the need to improve effective pathway and access to a PR center for JIA patients.

Vaccination completeness in children with rheumatic diseases: A longitudinal, observational multicenter cohort study in Switzerland.

Introduction: Children with pediatric inflammatory rheumatic diseases (PRD) have an increased infection risk. Vaccinations are effective to avoid vaccine-preventable diseases. This study aimed to assess the vaccination completeness in Swiss PRD patients stratified by immunosuppressive treatment (IST). Materials and methods: This multicenter observational cohort study of PRD patients was performed in Basel, Geneva, Lucerne, Lausanne, and Zurich in PRD patients aged < 18 years included in the Juvenile Inflammatory Rheumatism Cohort. Completeness was assessed for i) the overall vaccination status (Swiss national immunization program (NIP) and specific additional PRD-recommended vaccinations), ii) for all and each vaccination of the NIP at PRD diagnosis and reference date (RefD) and iii) all and each specific additional PRD-recommended vaccination at RefD. Completeness was assessed over the disease course and stratified by IST. Results: Of 616 eligible patients, 234 children were analyzed. Of these, 147 (63%) were girls. Median age at PRD diagnosis was 6.5 years (IQR 2.9-10.3) and 10.9 years at RefD (6.9-14.3). The median follow-up since PRD diagnosis was 3 years (1.1-5.5). 120/234 children received IST. At RefD, overall vaccination completeness was 3.8% (9/234 children), completeness for the NIP vaccinations was 70.1% (164/234 children; IST 65%, no IST: 75.4%) and for all specific additional PRD-recommended vaccinations was 3.8% (9/234 children; IST 2.5%; no IST 5.3%). Vaccination completeness against pneumococcal disease, hepatitis B virus, and human papilloma virus (HPV) was 50.4, 20, 37.9%, respectively. In 25/35 children with negative varicella zoster virus history vaccination status was complete (IST: 94.4%, no IST: 47%). Annual non-live influenza vaccination was complete in 24.2% of children during IST; adherence decreased over the disease course. Discussion: This study identified a low overall vaccination completeness in children with PRD. Particularly, the completeness of specific additional PRD-recommended vaccinations was low. If not performed early after PRD diagnosis, vaccination status remained frequently incomplete. Close collaboration between pediatrician and rheumatologist to improve vaccination completeness is essential. Exchange of vaccination records, standardized assessment of specific PRD-recommended vaccinations and those of the NIP, and annual reminder for influenza vaccination are crucial to improve vaccination completeness in this vulnerable pediatric population.

Schizophrenia Genetics and Neuropsychiatric Features in Childhood-onset Systemic Lupus Erythematosus.

OBJECTIVE: We examined the association between schizophrenia genetic susceptibility loci and neuropsychiatric systemic lupus erythematosus (NPSLE) features in childhood-onset SLE (cSLE) participants. METHODS: Study participants from the Lupus Clinic at the Hospital for Sick Children, Toronto, met ≥ 4 of the American College of Rheumatology and/or SLE International Collaborating Clinics SLE classification criteria and were genotyped using the Illumina Multi-Ethnic Global Array or the Global Screening Array. Ungenotyped single-nucleotide polymorphisms (SNPs) were imputed, and ancestry was genetically inferred. We calculated 2 additive schizophrenia-weighted polygenic risk scores (PRS) using (1) genome-wide significant SNPs (P < 5 × 10-8), and (2) an expanded list of SNPs with significance at P < 0.05. We defined 2 outcomes compared to absence of NPSLE features: (1) any NPSLE feature, and (2) subtypes of NPSLE features (psychosis and nonpsychosis NPSLE). We completed logistic and multinomial regressions, first adjusted for inferred ancestry only and then added for variables significantly associated with NPSLE in our cohort (P < 0.05). RESULTS: We included 513 participants with cSLE. Median age at diagnosis was 13.8 years (IQR 11.2-15.6), 83% were female, and 31% were of European ancestry. An increasing schizophrenia genome-wide association PRS was not associated with NPSLE (OR 1.04, 95% CI 0.87-1.26, P = 0.62), nor with the NPSLE subtypes, psychosis (OR 0.97, 95% CI 0.73-1.29, P = 0.84) and other nonpsychosis NPSLE (OR 1.08, 95% CI 0.88-1.34, P = 0.45), in ancestry-adjusted models. Results were similar for the model including covariates (ancestry, malar rash, oral/nasal ulcers, arthritis, lymphopenia, Coombs-positive hemolytic anemia, lupus anticoagulant, and anticardiolipin antibodies) and for the expanded PRS estimates. CONCLUSION: We did not observe an association between known risk loci for schizophrenia and NPSLE in a multiethnic cSLE cohort. This work warrants further validation.

Chronic Nonbacterial Osteomyelitis in Children.

Chronic nonbacterial osteomyelitis (CNO) is an auto-inflammatory bone disorder with a wide spectrum of clinical manifestations, from unifocal to multifocal lesions. When it manifests with multifocal lesions, it is also referred to as chronic recurrent multifocal osteomyelitis (CRMO). CNO/CRMO can affect all age groups, with the pediatric population being the most common. Patients may present with systemic inflammation, but there is no pathognomonic laboratory finding. Magnetic resonance imaging (MRI) is the gold standard radiological tool for diagnosis. In the absence of validated diagnostic criteria, CNO/CRMO remains an exclusion diagnosis. Bone biopsy does not show a specific disease pattern, but it may be necessary in unifocal or atypical cases to differentiate it from malignancy or infection. First-line treatments are non-steroidal anti-inflammatory drugs (NSAIDs), while bisphosphonates or TNF-α blockers can be used in refractory cases. The disease course is unpredictable, and uncontrolled lesions can complicate with bone fractures and deformations, underlying the importance of long-term follow-up in these patients.

Benefit of Temporomandibular Joint Lavage With Intra-Articular Steroids Versus Lavage Alone in the Management of Temporomandibular Joint Involvement in Juvenile Idiopathic Arthritis.

PURPOSE: The aim of the present investigation was to evaluate, in patients with juvenile idiopathic arthritis, the effect of lavage with or without intra-articular corticosteroid (IACS) injection on clinical temporomandibular joint (TMJ) signs and symptoms of inflammation and changes in acute inflammation as assessed using magnetic resonance imaging (MRI). MATERIALS AND METHODS: Forty-one patients (mean age, 13.6 ± 4.0 yr) with juvenile idiopathic arthritis participating in a large prospective juvenile inflammatory rheumatism cohort study (JIRcohorte) were included in this study. Clinical history, examination, and MRI were carried out at baseline and 6 months after intervention, if any. Twenty-one patients underwent lavage and IACS injection in at least 1 TMJ, 8 patients underwent lavage of at least 1 TMJ, and 12 patients were followed with no intervention. Outcomes measured were maximal mouth opening, Helkimo dysfunction index scores, pain intensity, and acute inflammation as assessed using MRI. RESULTS: All groups showed a mean increase in mouth opening and mean decrease in pain intensity. The mean Helkimo clinical dysfunction score decreased for the 2 intervention groups but not for the control group. The mean Helkimo anamnestic dysfunction score decreased for the lavage with IACS group but not for the lavage-only group. The only statistically relevant difference was found for the Helkimo anamnestic dysfunction score comparing the lavage-only with the lavage with IACS group, with a more positive effect found in the lavage with IACS group. More than 50% of joints in each group showed no change at MRI examination. Joints with lavage and ICAS injection showed better improvement than joints that had lavage only or no intervention. CONCLUSION: TMJ lavage with or without IACS injection cannot be claimed to systematically decrease pain, increase mouth opening, or resolve acute inflammation. Despite a tendency for improvement, response to this treatment is very patient dependent and can be determined by an array of other variables.

[Recurrent auto-inflammatory fevers : a practical diagnostic flow chart]. / Fièvres récurrentes auto-inflammatoires : la bonne démarche diagnostique.

Recurrent autoinflammatory fever syndromes are characterized by an abnormal activation of the innate immune system pathways, leading to inappropriate systemic inflammation responsible for clinical symptoms. The diagnosis of these conditions is difficult because of their low prevalence, but also because of their nonspecific clinical signs. The presence of clinical inflammatory signs such as fever associated to serositis (arthritis, peritonitis …) or recurrent cutaneous manifestations and elevated acute phase reactants should help the clinician to make the correct diagnosis. The purpose of this article is to provide a practical management flow chart for children with a suspected autoinflammatory recurrent fever syndrome.

Les fièvres récurrentes auto-inflammatoires sont un groupe de pathologies qui ont comme point commun une activation anormale du système immunitaire inné, en bonne partie liées à des mutations dans les gènes régulateurs des cascades inflammatoires. Le diagnostic de ces pathologies est difficile en raison de leur faible prévalence, mais aussi de leurs signes cliniques peu spécifiques. La présence de signes cliniques et biologiques inflammatoires tels que de la fièvre associée à une inflammation des séreuses (arthrite, péritonite…) et de signes cliniques cutanés récurrents doit faire évoquer le diagnostic et conduire à une analyse sémiologique fine afin de pouvoir poser un diagnostic précis. Cet article propose une conduite à tenir de pratique clinique courante devant un enfant atteint d'une fièvre à répétition.

[News in paediatrics]. / Pédiatrie.

Every pediatrician will be confronted with newborns oryoung infants with skin lesions in proximity of the vertebral column. It is important not to miss a spinal dysraphism because of the risk of meningeal infection or of the possible presence of a tethered cord. A practical algorithm is presented. Non-accidental injury in young infants and toddlers is not rare but difficult to detect. Bruises and fractures are highly suspicious for non-accidental injury and should trigger specific investigations. Emergency departments and hospitals are switching from hypotonic to isotonic solutions as maintenance infusions of children. They reduce the risk of hyponatremia without increasing that of hypernatremia, and they should be used preferentially in the majority of pediatric clinical settings.

Pneumococcal and influenza vaccination rates and their determinants in children with chronic medical conditions.

BACKGROUND: To investigate the rates of pneumococcal and influenza vaccinations and their determinants in children with chronic medical conditions. PATIENTS AND METHODS: Children with HIV infection, cystic fibrosis, liver transplantation and diabetes mellitus were enrolled. Physicians of regional Reference Centres for each condition, primary care paediatricians and caregivers of children provided information through specific questionnaires. For diabetes, 3 Reference Centres were included. RESULTS: Less than 25% of children in each group received pneumococcal vaccination. Vaccination rates against influenza were 73% in patients with HIV-infection, 90% in patients with cystic fibrosis, 76% in patients with liver transplantation, and ranged from 21% to 61% in patients with diabetes mellitus. Reference Centres rather than primary care paediatricians had a major role in promoting vaccinations. Lack of information was the main reason for missing vaccination. Awareness of the severity of pneumococcus infection by key informants of at-risk children was associated with higher vaccination rate. CONCLUSIONS: Vaccination rates in children with chronic conditions were poor for pneumococcus and slightly better for influenza. Barriers to vaccination include lack of awareness, health care and organization problems.