The German version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the German language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. The participating centres were asked to collect demographic and clinical data along the JAMAR questionnaire in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach\u2019s alpha, interscale correlations, test\u2013retest reliability, and construct validity (convergent and discriminant validity). A total of 319 JIA patients (2.8% systemic, 36.7% oligoarticular, 23.5% RF negative polyarthritis, and 37% other categories) and 100 healthy children were enrolled in eight centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the German version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and in clinical research

Imaging in juvenile idiopathic arthritis - international initiatives and ongoing work

Imaging is increasingly being integrated into clinical practice to improve diagnosis, disease control and outcome in children with juvenile idiopathic arthritis. Over the last decades several international groups have been launched to standardize and validate different imaging techniques. To enhance transparency and facilitate collaboration, we present an overview of ongoing initiatives

Onset of thymic recovery and plateau of thymic output are differentially regulated after stem cell transplantation in children

Die thymusabhängige T-Zellregeneration ist ein wichtiger Weg der Immunrekonstitution nach Stammzelltransplantation im Kindesalter. Aus diesem Grund wurde die Thymusfunktion sowie die T-Zelldynamik bei 164 pädiatrischen Patienten zwischen 1 und 124 Monaten nach Transplantation durch Messung der Exzisionsprodukte der TZellrezeptorgenumlagerung (TRECs) und der spontanen Ki67-Expression in peripheren T-Zellsubpopulationen prospektiv untersucht. Analysiert wurde der Einfluss des Empfängeralters, des Konditionierungsregimes, der Erkrankung, der Art des Spenders oder des Transplantates, der verwendeten Stammzellzahl und der GvHD auf den Beginn sowie auch auf das Plateau der Thymusauswurfleistung. Die Rate der spontanen Proliferationsaktivität von naiven und Gedächtnis-T-Zellen war mit der Gesamt-T-Zellzahl zum jeweiligen Zeitpunkt invers korreliert. Eine Multivariatanalyse zeigte, dass der Beginn der Wiederherstellung der Thymusfunktion allein mit dem Patientenalter (P < 0,0002) korrelierte. Das Plateau der thymischen Aktivität war jedoch bei Patienten, die eine größere Stammzelldosis erhalten hatten (> 107 CD34+/kg Körpergewicht), signifikant höher als bei solchen, denen nur weniger Stammzellen transplantiert werden konnten (P < 0,0022). Die anderen untersuchten Faktoren hatten keinen signifikanten Einfluss auf diese beiden Parameter. Demzufolge ist die durch Lymphopenie getriggerte Proliferation ein wichtiges Instrument der T- Zellhomöostase in der frühen Phase nach Stammzelltransplantation, kann aber zu einer Unterschätzung der Thymusfunktion führen. Beginn und Plateau der thymischen Aktivität sind unabhängig voneinander durch verschiedene transplantationsassoziierte Faktoren reguliert.Onset of thymic recovery and plateau of thymic output are differentially regulated after stem cell transplantation in childre

Results of a multinational survey regarding the diagnosis and treatment of temporomandibular joint involvement in juvenile idiopathic arthritis

BACKGROUND: Temporomandibular joint (TMJ) involvement occurs in up to 80% of patients with juvenile idiopathic arthritis (JIA). Currently there are no standardized procedures regarding diagnosis and treatment of this common complication of JIA. The aim of the study was to assess the current clinical practices in many countries regarding diagnosis and treatment of TMJ involvement in JIA. Pediatric rheumatologists were asked to fill out a survey with 8 items regarding diagnosis and treatment of TMJ involvement. The survey was distributed over the worldwide pediatric rheumatology electronic list-serve. Data was collected in an Excel spread sheet and analyzed using Excel software. FINDINGS: Eighty-seven centers responded to the survey between December 2009 and April 2010. All responding centers were actively screening for TMJ involvement. All centers were screening by physical exam, 85 (97%) by history, and 2 (3%) by imaging. Seventy-seven (88%) centers were screening at the first visit and 76 (87%) at each follow-up visit. If imaging was requested, 77% of the centers reported that they asked for MRI, 10% for ultrasound, 9% for CT and 33% for X-ray. The first line treatment of TMJ arthritis was a non-biologic DMARD in 36%, an NSAID in 33%, an intraarticular corticosteroid injection in 26%, and an anti-TNF agent in 5%. Overall, 57 (65%) of the centers were using intraarticular corticosteroid injections as treatment. CONCLUSIONS: TMJ arthritis is common among children with JIA. This survey shows that a wide array of diagnostic and therapeutic approaches is being employed for TMJ disease in 87 international centers. Due to this lack of agreement in how to diagnose and treat this JIA complication, we believe that an expert opinion/consensus statement regarding TMJ arthritis in JIA will likely benefit patients worldwide

Juvenile idiopathic arthritis - the role of imaging from a rheumatologist\u2019s perspective

Alongside recent advances in treatment strategies for juvenile idiopathic arthritis (JIA), paediatric rheumatologists have taken increasing interest in the use of imaging. Magnetic resonance imaging (MRI) and musculoskeletal ultrasound, by providing more detailed information on disease activity than clinical examination and conventional radiography (CR), have become helpful diagnostic and managerial tools. The growing skeleton, however, with changing appearances over time, is still challenging in the establishment of valid scoring systems for pathological changes. Defining child- and age-specific reference standards is therefore a highly prioritized issue. The aim of this article is to raise awareness among radiologists of the substantial role that imaging can play to optimize the management of JIA patients and to describe the state-of-the-art validation process of imaging as an outcome measure. A closer collaboration between radiologists and pediatric rheumatologists is crucial to define a scheduled workflow for imaging in JIA