Elvira Cannizzaro

Temporomandibular joint involvement in children with juvenile idiopathic arthritis.

Objective. To determine the rate of temporomandibular joint (TMJ) involvement and find factors associated with TMJ arthritis in a single-center cohort of patients with juvenile idiopathic arthritis (JIA). Methods. Retrospective analysis of all patients with JIA visiting the rheumatology clinic between January 1, 2005, and December 31, 2006. Followup information was included until August 2008. A diagnosis of TMJ arthritis was based on clinical rheumatological and/or radiological findings. Results. After a mean followup time for JIA of 4.6 years (range 0.08–14.17), 86/223 patients (38.6%) had developed TMJ arthritis. The rate of TMJ involvement differed significantly among JIA subtypes (p = 0.0016), with 61% in extended oligoarticular, 52% in polyarticular rheumatoid factor (RF)-negative, 50% in psoriatic, 36% in systemic, 33% in polyarticular RF-positive, 33% in persistent oligoarticular, 30% in unclassified JIA, and 11% in enthesitis-related arthritis. The rate of TMJ involvement in our cohort was statistically significantly lower for patients who were HLA-B27-positive (p = 0.0002). In a multivariate analysis, the association of the following factors was confirmed: JIA subtype (p = 0.0001), a higher erythrocyte sedimentation rate (ESR) at diagnosis (p = 0.0038), involvement of joints of the upper extremity (p = 0.011), the absence of HLA-B27 (p = 0.023), and younger age at onset of JIA (p = 0.050). Conclusion. In our cohort of children with JIA, the overall rate of TMJ involvement was 38.6%. Patients with certain JIA subtypes, a higher ESR at disease onset, involvement of upper extremity joints, and younger age at diagnosis were more likely to develop TMJ arthritis. The presence of HLA-B27 seemed to be protective.

A 12-year-old girl with absent radial pulse: arterial thoracic outlet syndrome with subclavian artery aneurysm and thrombosis of the brachial artery

Brachial arterial occlusion is rare in children and adolescents. Once a traumatic cause is excluded, the differential diagnosis consists of a variety of rare conditions. We report the case of a 12-year-old girl whose presenting symptoms—an absent radial pulse and Raynaud’s phenomenon of the right hand—could be easily mistaken for a vasculitis. She was found to have arterial thoracic outlet syndrome with right subclavian artery compression and aneurysm formation caused by an anomalous first rib and consecutive thromboembolic occlusion of the brachial artery. The diagnosis and differential diagnosis of this condition are reviewed.

Temporomandibular joint arthritis in patients with juvenile idiopathic arthritis: efficacy of intraarticular corticosteroid injection as measured by MRI and clinical examination

Results 21 study patients and 17 control patients were examined. The baseline mean maximal mouth opening was significantly different with 41 mm in study patients compared to 46 mm in controls (p = 0.005). After a median time of 42 days the mean maximal mouth opening increased by 1.8 mm in the study group (p < 0.003) as compared to 0.5 mm in the controls (p = 0.15). Pain on chewing/yawning had resolved in all 5 patients and tenderness in 7/11 TMJs respectively. On follow up MRI 23/36 affected joints showed improvement and 6/36 complete resolution of inflammation. Conclusion In our JIA patients with MRI proven active TMJ arthritis intraarticular steroid injection led to resolution of clinical symptoms and significantly improved mouth opening in most patients. However, MRI examination showed only improvement but not complete resolution of inflammation in the majority of patients. Longer follow up is warranted to assess the significance of persistent MRI changes for the mandibular growth in our patients. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Safety of biological agents in paediatric rheumatic diseases: A real-life multicenter retrospective study using the JIRcohorte database

OBJECTIVE To analyse and report the incidence of side effects of biological agents in paediatric patients with inflammatory diseases using of real-life follow-up cohort. METHODS In this international, observational, retrospective, multicentre study of children treated by biological agents and followed in the Juvenile Inflammatory Rheumatism (JIR) cohort (JIRcohorte) network, a Kaplan-Meier method was used to estimate the occurrence of adverse events. A Cox model was constructed to identify independent predictors of adverse events. RESULTS Overall 813 patients totalling 3439 patients-year (PY) of biological agents were included. The main diagnosis was juvenile idiopathic arthritis (84%). A total of 222 patients (27.3%) had 419 adverse events, representing an incidence rate of 12.2 per 100 PY 95% CI [11.0; 13.4]. The overall incidence rate of serious adverse events was 3.9 per 100 PY 95% CI [3.2; 4.6]. Tocilizumab and infliximab were significantly associated with adverse events and canakinumab with serious adverse events. Univariate and multivariable analysis of adverse events and serious adverse events indicated that patients under biological agents with concomitant immunosuppressive drugs (excluding methotrexate) suffered from more of these events. CONCLUSION This study suggests an overall an acceptable safety of biologic agents in children with inflammatory rheumatic diseases treated with biological agents. However, the concomitant prescription of immunosuppressive drugs with biological agents represents a substantial risk of adverse events.

Multicenter retrospective study of biological tolerance in juvenile idiopathic arthritis (jir-cohort)

Ten years after the introduction of biologics in children, we assess the tolerance of these therapies among patients with juvenile idiopathic arthritis (JIA) in pediatric rheumatology centers.

Switch of biotherapies in patients with juvenile idiopathic arthritis: analyses of the JIR cohort data

Biologic treatments have been introduced for Juvenile Idiopathic Arthritis (JIA) treatment in 2000, and have substantially improved the global prognosis of all disease subtypes. However not all patients respond to one biologic and therapeutic effect of one drug may decrease with time.

Prescribed but not approved: biologic agents used without approval in juvenile idiopathic arthritis in Switzerland, France and Belgium

Biologic agents (BA) have profoundly changed the outcome of juvenile idiopathic arthritis (JIA), making inactive disease and clinical remission an achievable goal for treatment. An increasing number of BA has become available in the last 15 years. However, some BA that have been associated to efficacy in some clinical conditions are not approved by legal authority for the use in pediatric population.