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The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to update the current guidelines, and the objective here was to produce this document to guide clinicians managing children with juvenile idiopathic arthritis–associated uveitis (JIAU) and idiopathic chronic anterior uveitis (CAU). The group analyzed the literature published between December 2014 and June 2020 after a systematic literature review conducted by 2 clinicians. Pediatric rheumatologists were paired with ophthalmologists to review the eligible 37 publications. The search criteria were selected to reflect those used for the 2018 Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) recommendations, in order to provide an update, rather than a replacement for that publication. The summary of the current evidence for each SHARE recommendation was presented to the expert committee. These recommendations were then discussed and revised during a video consensus meeting on January 22, 2021, with 14 voting participants, using a nominal group technique to reach consensus. JIAU treatment was extended to include CAU. Fourteen recommendations regarding treatment of JIAU und CAU with >90% agreement were accepted. An update to the previous 2018 SHARE recommendations for the treatment of children with JIAU with the addition of CAU was created using an evidence‐based consensus process. This guideline should help support clinicians to care for children and young people with CAU.

To determine the relative importance weights of items and grades of a newly developed additive outcome measure called the juvenile idiopathic arthritis (JIA) magnetic resonance imaging (MRI) scoring system for the temporomandibular joint (TMJ) (JAMRIS‐TMJ). An adaptive partial‐profile, discrete choice experiment (DCE) survey using the 1000Minds platform was independently completed by members of an expert group consisting of radiologists and non‐radiologist clinicians to determine the group‐averaged relative weights for the JAMRIS‐TMJ. Subsequently, an image‐based vignette ranking exercise was done, during which experts individually rank ordered 14 patient vignettes for disease severity while blinded to the weights and unrestricted to JAMRIS‐TMJ assessment criteria. Validity of the weighted JAMRIS‐TMJ was tested by comparing the consensus‐graded, DCE‐weighted JAMRIS‐TMJ score of the vignettes with their unrestricted image‐based ranks provided by the experts. Nineteen experts completed the DCE survey, and 21 completed the vignette ranking exercise. Synovial thickening and joint enhancement showed higher weights per raw score compared to bone marrow items and effusion in the inflammatory domain, while erosions and condylar flattening showed nonlinear and higher weights compared to disk abnormalities in the damage domain. The weighted JAMRIS‐TMJ score of the vignettes correlated highly with the ranks from the unrestricted comparison method, with median Spearman's ρ of 0.92 (interquartile range [IQR] 0.87–0.95) for the inflammation and 0.93 (IQR 0.90–0.94) for the damage domain. A DCE survey was used to quantify the importance weights of the items and grades of the JAMRIS‐TMJ. The weighted score showed high convergent validity with an unrestricted, holistic vignette ranking method.

To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status.

The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJpathology on magnetic resonance imaging (MRI). Consensus‐driven development and validation of an MRIscoring system for TMJs has important clinical utility in timely improvement of diagnosis and serving as an outcome measure. We report on a multi‐institutional collaboration toward developing a TMJ MRIscoring system for JIA. Seven readers independently assessed MRIscans from 21 patients (42 TMJs, from patients ages 6–16 years) using 3 existing MRIscoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions, and items were discussed among 10 JIAexperts through 2 rounds of Delphi surveys, nominal group voting, and subsequent consensus meetings to create a novel TMJ MRIscoring system. Average‐measure absolute agreement intraclass correlation coefficients (avICCs) for the total scores of all 3 scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed preferable for assessing minor joint changes over time. Eight items were considered sufficiently reliable and/or important for integration into the consensus scoring system: bone marrow edema and enhancement (avICC0.57–0.61, smallest detectable difference [SDD] ± 45–63% prior to redefining), condylar flattening (avICC0.95–0.96, SDD± 23–28%), effusions (avICC0.85–0.88, SDD± 25–26%), erosions (avICC0.94, SDD± 20%), synovial enhancement and thickening (previously combined, avICC0.90–0.91, SDD± 33%), and disk abnormalities (avICC0.90, SDD± 19%). A novel TMJ MRIscoring system was developed by consensus. Further iterative refinements and reliability testing are warranted in upcoming studies.

To evaluate the demographic, disease activity, disability, and health‐related quality of life (HRQOL) differences between children with juvenile idiopathic arthritis (JIA) and their healthy peers, and between children with JIA with and without clinical temporomandibular joint (TMJ) involvement and its determinants. This study is based on a cross‐sectional cohort of 3,343 children with JIA and 3,409 healthy peers, enrolled in the Pediatric Rheumatology International Trials Organisation HRQOL study or in the methotrexate trial. Potential determinants of TMJ involvement included demographic, disease activity, disability, and HRQOL measures selected through univariate and multivariable logistic regression. Clinical TMJ involvement was observed in 387 of 3,343 children with JIA (11.6%). Children with TMJ involvement, compared to those without, more often had polyarticular disease course (95% versus 70%), higher Juvenile Arthritis Disease Activity Score (odds ratio [OR] 4.6), more disability, and lower HRQOL. Children with TMJ involvement experienced clearly more disability and lower HRQOL compared to their healthy peers. The multivariable analysis showed that cervical spine involvement (OR 4.6), disease duration >4.4 years (OR 2.8), and having more disability (Childhood Health Assessment Questionnaire Disability Index >0.625) (OR 1.6) were the most important determinants for TMJ involvement. Clinical TMJ involvement in JIA is associated with higher disease activity, higher disability, and impaired HRQOL. Our findings indicate the need for dedicated clinical and imaging evaluation of TMJ arthritis, especially in children with cervical spine involvement, polyarticular course, and longer disease duration.

Lumbalgien sind im Erwachsenenalter ein weit verbreitetes Phänomen, die meisten Erwachsenen sind irgendwann in ihrem Leben einmal davon betroffen [1]. Für den Rheumatologen auf der Notfallstation sind Rückenschmerzen bei unter 20-Jährigen ein „red flag“, wie wir im vorhergehenden Artikel dieser Ausgabe lesen konnten (Marcel Weber: Rückenschmerzen im Notfall). Wie sieht die Situation also aus im Kindes- und Jugendalter?

ObjectiveJuvenile idiopathic arthritis JIA is a heterogeneous disease involving chronic arthritis. The clinical course is characterized by a fluctuating pattern of active and inactive disease. We have described in detail the clinical course in different JIA subtypes during the first 2 years after diagnosis and studied its relationship to disease activity in the following years.MethodsDetailed clinical data on different parameters describing the disease activity in sequential time periods covering the first 2 years after diagnosis were retrieved from the charts of 311 patients with JIA and compared between subtypes. In a cohort of 146 patients, the relation of these different clinical variables to the course of disease in the following 3 years was evaluated.ResultsThe percentage of time with active disease in the first 2 years differed significantly between subtypes. In all subtypes, a broad spectrum of activity was observed. The time with active disease in the first 2 years was the most significant factor associated with the duration of active disease in the following years.ConclusionDifferent percentages of time with active disease have been observed between JIA subtypes in the first 2 years. The cumulative duration of activity varied widely within each subtype. Regarding the prognosis of the individual patient, the clinical course in the first 2 years appears to be predictive of the clinical course in the following years. Patients that have less time with active disease in the first 2 years are not likely to develop an unremitting clinical course later on.

OBJECTIVE: To compare the clinical features and outcome between patients with juvenile psoriatic arthritis (JPsA) and non-JPsA juvenile idiopathic arthritis (JIA). METHODS: Fifty-three children with JPsA, 32 with < 5 joints in the first 6 months of disease (oligo–JPsA) and 21 (≥ 5 joints) polyarticular-onset (poly-JPsA) were compared to 53 patients with JIA who were matched by sex, age, date of diagnosis, and articular onset pattern. RESULTS: There was no difference in the percentage of patients between the oligoarticular groups who developed extended oligoarthritis or in the percentage of patients who were positive for antinuclear antibodies. The only differences were that 25% of patients with oligo-JPsA had dactylitis compared to 0% of patients with oligo-JIA (p < 0.01) and 50% had nail pitting as compared to 18.7% (p < 0.05). In polyarticular patients the percentages with dactylitis were similar (19% vs 38%; p = 0.25). The frequency of uveitis was identical in the oligoarticular patients but a higher rate was seen in poly-JPsA compared to poly-JIA (23.8% vs 0%; p = 0.02), while contractures were more frequent in poly-JIA compared to poly-JPsA during the course of the illness (47.6% vs 14.3%; p = 0.03) but not at last followup (14.3% vs 4.7%; p = 0.6). At last followup the mean Childhood Health Assessment Questionnaire scores were similar in both the polyarticular and oligoarticular groups. CONCLUSION: There were only a few differences between patients with JPsA and JIA regarding disease onset, disease course, and outcome. We suggest that large, longterm prospective studies are required to accurately determine whether subdividing JIA according to psoriasis is worthwhile.

To validate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile dermatomyositis (DM).In 2001, a preliminary consensus‐derived core set for evaluating response to therapy in juvenile DM was established. In the present study, the core set was validated through an evidence‐based, large‐scale data collection that led to the enrollment of 294 patients from 36 countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, concordance in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response.The following clinical measures were found to be feasible, and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) muscle strength, 3) global disease activity measure, 4) parent's global assessment of patient's well‐being, 5) functional ability, and 6) health‐related quality of life.The members of the Paediatric Rheumatology International Trials Organisation, with the endorsement of the American College of Rheumatology and the European League Against Rheumatism, propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a child with juvenile DM has responded adequately to therapy.

OBJECTIVE: Uveitis occurs in 10%-15% of patients with juvenile idiopathic arthritis (JIA). If topical treatment fails, second-line agents are used to control the disease. However, some patients need the addition of tumor necrosis factor-a (TNF-a) antagonist (anti-TNF). We organized a cross-sectional cohort to investigate use and efficacy of anti-TNF treatment in patients with JIA-associated uveitis. METHODS: The international pediatric rheumatology community was queried about the use and efficacy of anti-TNF in treatment of JIA-associated uveitis using an E-mail survey. RESULTS: Of the 33 responding centers following 884 patients with uveitis, only 15 centers, following 404 patients, were using anti-TNF for this indication. A total of 47 patients with JIA-related uveitis treated with anti-TNF because of an insufficient response to previous therapy were reported. The mean age of the patients was 12.5 years. The mean duration from onset of uveitis to start of anti-TNF treatment was 45.1 months. Three different anti-TNF agents were used: etanercept in 34 cases, infliximab in 25 cases, and adalimumab in 3 cases. In 12 of the 34 patients etanercept was inefficacious and patients were switched to infliximab. The final response was rated according to a composite index as 53%/12%/32%, and according to physician rating as 47%/12%/38% representing good, moderate, and poor, respectively, in the etanercept group; and 70%/30%/0% and 68%/24%/0% in the infliximab group. All 3 patients taking adalimumab were responders. Infliximab was statistically significantly more efficacious for the treatment of JIA-associated uveitis than etanercept (chi-square p = 0.004). CONCLUSION: Anti-TNF seems to be an effective treatment for refractory JIA-associated uveitis. In this cohort infliximab was more efficacious than etanercept.

Thrombolytic therapy is a well-defined treatment option for arterial and venous thrombosis in adults. In contrast, uniform recommendations regarding the indication, route of administration, and dosing of thrombolytic therapy in children are not available. The authors report the successful resolution of bilateral pulmonary embolism and popliteal artery thrombosis in an 11-year-old girl and 13-year-old girl, respectively, by catheter-directed thrombolysis with low-dose recombinant tissue plasminogen activator. Catheter-directed low-dose thrombolysis is an efficient treatment option for severe venous and arterial thrombosis in children.

ZusammenfassungDie axilläre Lymphonodektomie ist immer noch integraler Bestandteil der Mammakarzinomtherapie, verursacht aber eine deutliche Morbidität. Die Sentinel-Lymphknotenoperation weist eine wesentlich geringere Morbidität auf und vermeidet bei gegebener Indikation zunehmend die komplette Axilladissektion. Die Sentinel-Lymphonodektomie hat sich als neuer operativer Standard etabliert und sollte allen Patientinnen mit unizentrischen Tumoren unter 3 cm Durchmesser angeboten werden. Die offenen Fragen betreffen vor allem den Injektionsort der Markersubstanz, die Bedeutung des Auffindens und Entfernens extra-axillärer Lymphknoten und die Anwendbarkeit der Methode bei multizentrischem Tumor, nach neoadjuvanter Chemotherapie, vorausgegangener Brustbiopsie, beim DCIS und bei Tumoren von mehr als 3 cm Grösse. Auch das Vorgehen nach Auffinden von Mikrometastasen ist noch Gegenstand wissenschaftlicher Studien.

Die axilläre Lymphonodektomie ist immer noch integraler Bestandteil der Mammakarzinomtherapie, verursacht aber eine deutliche Morbidität. Die Sentinel-Lymphknotenoperation weist eine wesentlich geringere Morbidität auf und vermeidet bei gegebener Indikation zunehmend die komplette Axilladissektion. Die Sentinel-Lymphonodektomie hat sich als neuer operativer Standard etabliert und sollte allen Patientinnen mit unizentrischen Tumoren unter 3 cm Durchmesser angeboten werden. Die offenen Fragen betreffen vor allem den Injektionsort der Markersubstanz, die Bedeutung des Auffindens und Entfernens extra-axillärer Lymphknoten und die Anwendbarkeit der Methode bei multizentrischem Tumor, nach neoadjuvanter Chemotherapie, vorausgegangener Brustbiopsie, beim DCIS und bei Tumoren von mehr als 3 cm Grösse. Auch das Vorgehen nach Auffinden von Mikrometastasen ist noch Gegenstand wissenschaftlicher Studien.