Silke Schroeder

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.

Infliximab in pediatric rheumatology patients: a retrospective analysis of infusion reactions and severe adverse events during 2246 infusions over 12 years.

Objective. To describe infusion reactions (IR) and severe adverse events (SAE) associated with infliximab (IFX) in pediatric patients with rheumatologic and ocular inflammatory diseases in a real-world setting. Methods. This is a retrospective chart review of all patients treated with IFX at the pediatric rheumatology division of a university hospital between October 2000 and December 2012. Results. A total of 2446 IFX infusions were given to 82 patients (72% female). IR occurred in 46 infusions (2%) of 14 patients (17%) after a mean IFX treatment time of 340 days (range 41–780); 9/14 patients (64%) experienced repeated IR. IR were classified as mild (26%), moderate (74%), or severe (0%). Indications for IFX were arthritis (60%), uveitis (20%), arthritis and uveitis (13%), and other inflammatory diseases (5%). The most common clinical symptoms were respiratory signs (72%), cutaneous manifestations (69%), and malaise (61%). In 6/14 patients (43%) with IR, IFX was discontinued: 4 patients because of repeated IR and 2 patients wished to stop treatment immediately following a mild IR. The other 8/14 patients (57%) received premedication with high-dose antihistamine (100%), corticosteroids (75%), and IFX dose increase (75%) and continued IFX treatment for a mean followup period of 146 weeks (range 26–537) after the first IR. We observed severe infections in 5/82 patients (6%); other SAE were rare. Conclusion. Mild and moderate IR occurred in 17% of our patients. Treatment with antihistamines and methylprednisolone, and increasing the IFX dose, allowed continued treatment despite IR in > 50% of patients. Other SAE were infrequent.

A5: Detectable Anti-Infliximab Antibodies in Children Treated with Infliximab for Rheumatic Diseases

Infliximab (IFX) is a monoclonal TNF‐alpha inhibiting antibody which is frequently used to treat children with refractory arthritis and uveitis. The most frequent and limiting adverse events are infusion reactions associated with the presence of anti‐drug antibodies.

Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: an international collaboration

BackgroundRheumatic diseases in children are associated with significant morbidity andpoor health-related quality of life (HRQOL). There is no health-relatedquality of life (HRQOL) scale available specifically for children with lesscommon rheumatic diseases. These diseases share several features withsystemic lupus erythematosus (SLE) such as their chronic episodic nature,multi-systemic involvement, and the need for immunosuppressive medications.HRQOL scale developed for pediatric SLE will likely be applicable tochildren with systemic inflammatory diseases.FindingsWe adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters(SMILEY©) to Simple Measure of Impact of Illness in Youngsters(SMILY©-Illness) and had it reviewed by pediatric rheumatologists forits appropriateness and cultural suitability. We tested SMILY©-Illnessin patients with inflammatory rheumatic diseases and then translated it into28 languages.Nineteen children (79% female, n=15) and 17 parents participated. The meanage was 12±4 years, with median disease duration of 21 months (1-172months). We translated SMILY©-Illness into the following 28 languages:Danish, Dutch, French (France), English (UK), German (Germany), German(Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil),Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish(Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans,Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian,Japanese, Romanian, Serbian and Xhosa.ConclusionSMILY©-Illness is a brief, easy to administer and score HRQOL scale forchildren with systemic rheumatic diseases. It is suitable for use acrossdifferent age groups and literacy levels. SMILY©-Illness with itsavailable translations may be used as useful adjuncts to clinical practiceand research.

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

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.