Preliminary definitions for the sonographic features of synovitis in children

Objectives Musculoskeletal ultrasonography (US) has the potential to be an important tool in the assessment of disease activity in childhood arthritides. To assess pathology, clear definitions for synovitis need to be developed first. The aim of this study was to develop and validate these definitions through an international consensus process. Methods The decision on which US techniques to use, the components to be included in the definitions as well as the final wording were developed by 31 ultrasound experts in a consensus process. A Likert scale of 1-5 with 1 indicating complete disagreement and 5 complete agreement was used. A minimum of 80% of the experts scoring 4 or 5 was required for final approval. The definitions were then validated on 120 standardized US images of the wrist, MCP and tibiotalar joints displaying various degrees of synovitis at various ages. Results B-Mode and Doppler should be used for assessing synovitis in children. A US definition of the various components (i.e. synovial hypertrophy, effusion and Doppler signal within the synovium) was developed. The definition was validated on still images with a median of 89% (range 80-100) of participants scoring it as 4 or 5 on a Likert scale. Conclusions US definitions of synovitis and its elementary components covering the entire pediatric age range were successfully developed through a Delphi process and validated in a web-based still images exercise. These results provide the basis for the standardized US assessment of synovitis in clinical practice and research

Amendment of the OMERACT ultrasound definitions of joints' features in healthy children when using the DOPPLER technique

Background: Recently preliminary ultrasonography (US) definitions, in B mode, for normal components of pediatric joints have been developed by the OMERACT US group. The aim of the current study was to include Doppler findings in the evaluation and definition of normal joint features that can be visualized in healthy children at different age groups. Methods: A multistep approach was used. Firstly, new additional definitions of joint components were proposed during an expert meeting. In the second step, these definitions, along with the preliminary B-mode-US definitions, were tested for feasibility in an exercise in healthy children at different age groups. In the last step, a larger panel of US experts were invited to join a web-based consensus process in order to approve the developed definitions using the Delphi methodology. A Likert scale of 1-5 was used to assess agreement. Results: Physiological vascularity and fat pad tissue were identified and tested as two additional joint components in healthy children. Since physiological vascularity changes over the time in the growing skeleton, the final definition of Doppler findings comprised separate statements instead of a single full definition. A total of seven statements was developed and included in a written Delphi questionnaire to define and validate the new components. The final definitions for fat pad and physiological vascularity agreed by the group of experts reached 92.9% and 100% agreement respectively in a web survey. Conclusion: The inclusion of these two additional joints components which are linked to detection of Doppler signal in pediatric healthy joints will improve the identification of abnormalities in children with joint pathologies

Ultrasound imaging in paediatric rheumatology

The role of ultrasound imaging in the diagnosis and monitoring of paediatric rheumatic diseases with special emphasis on recent scientific work regarding the evidence base and standardization of this technique is being reviewed. An overview of the most important practical aspects for the use of musculoskeletal ultrasound in a clinical setting is also provided. Huge scientific efforts and advances in recent years illustrate the increasing importance of musculoskeletal ultrasound in pediatric rheumatology. Several studies focused on setting an evidence-based standard for the ultrasound appearance of healthy and normal joints in children of all age groups. Physiologic vascularization and ossification were two main aspects of these studies. Other publications demonstrate that ultrasound imaging is also an important and useful tool to detect pathology as synovitis, tenosynovitis or enthesitis in children and to monitor pediatric patients with rheumatic conditions. Important practical aspects include training in the use of correct ultrasound techniques, as well as knowledge and experience of normal pediatric sonoanatomy and the appearance of pathological findings on ultrasound

Safety and efficacy of etanercept in children with juvenile idiopathic arthritis below the age of 2 years

Etanercept is approved for the treatment of patients with juvenile idiopathic arthritis (JIA) above the age of 2 years. Experience with younger children is limited. The aim of the present study was to evaluate the efficacy and safety of treatment with etanercept in children with JIA younger than 2 years. The prospective long-term observational BIKER registry documents baseline demographics, clinical characteristics, disease activity parameters and safety issues. Efficacy was determined using the PedACR response criteria, the JADAS-10 and the proposed criteria for inactive disease and remission after 3, 6, 12, 18 and 24 months. Safety assessments were based on adverse events (AE) and serious adverse events (SAEs) reports. Between January 2001 and June 2013, a total of 13 patients including four patients with systemic JIA (sJIA), four patients with extended oligoarthritis, one patient with persistent oligoarthritis and four patients with RF negative polyarthritis were treated with etanercept. Eleven patients with follow-up assessments were analysed in our study. Prior to etanercept, all patients have been exposed to methotrexate. At last observation, 6/11 patients reached a PedACR 70 response. Two patients with sJIA and 1 with nonsystemic JIA achieved inactive disease. Tolerability was good in most of the patients. Eight AE and one SAE occurred. One patient with sJIA was affected by Hodgkin's disease 18 months after discontinuation of etanercept. New onset uveitis occurred in two patients. Reasons for discontinuation were inefficacy in three (2 sJIA), intolerance in two, remission in three (2 sJIA) and the parents' request in one patient. Etanercept seems to improve JIA patients younger than 2 years including some of the patients with sJIA. Attention should be paid to the development of malignancies and autoimmune disorders

Safety and efficacy of etanercept in children with the JIA categories extended oligoarthritis, enthesitis-related arthritis and psoriasis arthritis

The approval of etanercept for the treatment of the juvenile idiopathic arthritis (JIA) categories extended oligoarthritis (ExtOA), enthesitis-related arthritis (ERA) and psoriasis arthritis (PsA) was recently added to the approval for the treatment of polyarticular-course JIA (PA). This study aims to evaluate the efficacy and safety of etanercept in a large number of patients with the additional JIA categories. The Biologika in der Kinderrheumatologie (BIKER) registry documents baseline demographics, clinical characteristics and disease activity parameters. Efficacy was determined using the PedACR 30/50/70 response criteria and the Juvenile Arthritis Disease Activity Score (JADAS)-10. Safety assessments were based on adverse events' reports. Until December 2012, a total of 1,678 JIA patients, incorporating 238 ERA, 315 ExtOA and 127 PsA patients were included. JADAS-10 demonstrated marked improvement compared to baseline after 3 to 24 months in ExtOA [16.1 +/- 7.6 (baseline), 5.1 +/- 5.2 (3 months), 3.0 +/- 3.5 (24 months)]; ERA (15.3 +/- 7.2, 4.4 +/- 4.7, 4.0 +/- 4.9) and PsA (14.7 +/- 6.4, 5.0 +/- 4.6, 5.3 +/- 6.4). Compared to patients with PA, the rate of serious adverse events [relative risk (RR) 1.39 (0.95-2.03, p = 0.08)] and nonserious [1.18 (1.02-1.35; p = 0.03)] adverse events were elevated. The rate of uveitis flares was significantly higher in PsA (3.3/100 patient-years), ExtOA (2.8/100 patient-years) and ERA (2.7/100 patient-years) than in rheumatoid factor (RF)-negative polyarticular JIA (1.3/100 pat.yrs) or RF-positive polyarticular JIA (0.27/100 patient-years). Reports on chronic inflammatory bowel disease were numerically more frequent in ExtOA, ERA and PsA. Administration of etanercept in patients with the JIA categories ExtOA, ERA and PsA is safe and very efficacious in children. Attention should be paid to the occurrence of extraarticular autoimmunopathies

Cushing-Syndrom mit konsekutiver tertiärer Nebennierenrindeninsuffizienz nach simultaner multipler intraartikulärer Lokaltherapie mit Glukokortikoiden

After simultaneous multiple local treatment with glucocorticoids at 46 sites a 4‑year-old female patient with newly diagnosed polyarticular juvenile idiopathic arthritis (JIA) initially developed Cushing's syndrome followed by a gradual worsening of her condition and finally an acute high fever urinary tract infection. Iatrogenic adrenocortical insufficiency after multiple intra-articular glucocorticoid administration was diagnosed. The possibility of severe systemic glucocorticoid side effects after extensive local treatment should be included in the regular management of JIA patients

Current news from the BIKER register

Innovative developments in the pharmacotherapy of juvenile idiopathic arthritis and especially biologics allow the formulation of new therapeutic targets, such as the rapid induction of remission with shortening of the period of active disease and therefore preventing damage and disability. These new therapies also represent a challenge to the monitoring of drug safety, the pharmacovigilance. For this purpose the Society for Paediatric and Adolescent Rheumatology has set up an early register to record achievements in treatment improvement and in addition to independently assess information on drug safety, acute tolerance and long-term safety