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Das muskuloskeletale System unterliegt in Kindheit und Jugend bis zum Erreichen der Endgrose einem ausgepragten Langenwachstum. Zusatzlich muss sich das muskuloskeletale System kontinuierlich an die aktuellen auf Muskel und Knochen einwirkenden Beanspruchungen adaptieren. Uber die funktionelle Muskel-Knochen-Einheit wird im Sinne eines Regelkreises der Knochenaufbau und -abbau angepasst. Im Rahmen chronischer Erkrankungen (neurologische Erkrankungen, Knochenstoffwechselstorungen, rheumatisch-entzundliche Erkrankungen) aber auch im Rahmen einer kurzfristigen Immobilitat kommt es haufig direkt oder indirekt zu Veranderungen der funktionellen Muskel-Knochen-Einheit, der Gelenke oder der Bewegungsmuster, was letztlich in einer „Bewegungsstorung“ bzw. in veranderten Bewegungsmustern mundet. Unabhangig von der zugrunde liegenden Ursache scheinen die reduzierte Beweglichkeit, das veranderte Bewegungsmuster und die teilweise resultierende Immobilitat die Teilhabe der Betroffenen am Leben deutlich zu reduzieren, weswegen diesem Symptom eine hohe Wichtigkeit aus Sicht der Patienten zugeschrieben wird. Eine gezielte Diagnostik mit funktioneller Analyse der vorliegenden Bewegungsstorung erscheint unabdingbar, um ein interdisziplinar individuelles Betreuungskonzept, basierend auf der zugrunde liegenden Erkrankung, anbieten und dessen Wirksamkeit beurteilen zu konnen sowie die Lebensqualitat und Teilhabe der Betroffenen zu erhalten.

Entzundlich-rheumatische Erkrankungen im Kindes- und Jugendalter stellen in der Versorgung von akuten Traumata eine besondere Herausforderung dar. Bei Kindern und Jugendlichen haben sich die medikamentosen Behandlungsstrategien modifiziert. Welche Besonderheiten sind beim jungen Rheumatiker zu berucksichtigen, wenn ein Trauma zu einem operativen Verfahren zwingt? Uber eine Literaturrecherche werden Empfehlungen fur die Praxis erarbeitet. Die gelenkbetonten Veranderungen beim jungen Rheumatiker unterscheiden sich im Hinblick auf die unterschiedlich veranderten entzundlich-rheumatischen Destruktionen. Das Ausmas der entzundlich-destruktiven Veranderungen diktiert das operative Vorgehen. Konsequenzen ergeben sich in der Beachtung der Begleitmedikation im Hinblick auf die Vermeidung eines schubauslosenden Geschehens und einer Gewebeinfektion. Die Knochenfestigkeit zwingt zur individuellen Wahl von Implantaten und ggf. Dauer der Nachbehandlung. In den fruhen Stadien des Entzundungsprozesses unterscheiden sich die Vorgehensweisen bei Traumata nicht vom Gesunden, in spateren (Larsen-Dale-Eeck-Stadium III) schon. Ein interdisziplinares Konzept kann Nachteile in der Therapie der Grunderkrankung vermeiden helfen. Aufgrund der besonderen dysplastischen Anatomie und Gewebeveranderungen stellen Traumata bei dieser Klientel eine besondere Herausforderung dar.

The treatment of juvenile idiopathic arthritis (JIA) has made substantial progress within the last 25 years. Modern medicinal treatment enables inflammatory activity of the disease to be controlled in most of the cases. Mutilating courses of disease, which were formerly the rule have now become the exception. Today remission of disease is the aim of pediatric rheumatological treatment. Apart from effective control of inflammation this includes complete restoration of functional abilities of affected joints and the surrounding structures also affected. To achieve this goal a holistic and foresighted view of each patient's course is required. Therefore, even in an apparently uncomplicated course of disease in some cases of JIA it is advisable to plan an early interdisciplinary consultation including the pediatric rheumatologist and the orthopedic surgeon, in order to discuss an early surgical intervention, which can then be carried out in a timely manner, if necessary. This article provides an overview of the orthopedic rheumatological indications and options.Die Behandlung der juvenilen idiopathischen Arthritis (JIA) hat in den vergangenen 25 Jahren dramatische Fortschritte gemacht. Durch moderne medikamentöse Therapie lässt sich die Entzündungsaktivität in den meisten Fällen erfolgreich kontrollieren. Mutilierende Verläufe, wie sie früher regelhaft zu sehen waren, sind die Ausnahme geworden. Ziel einer kinderrheumatologischen Therapie ist heute die Remission. Dazu gehört neben einer erfolgreichen Kontrolle der entzündlichen Aktivität auch die vollständige funktionelle Wiederherstellung der betroffenen Gelenke und der gelenknahen, mitbetroffenen Strukturen. Dies erfordert eine ganzheitliche, vorausschauende Betrachtung des Erkrankungsverlaufes. Daher ist es auch bei scheinbar unkompliziertem Verlauf in einigen Fällen einer JIA ratsam, frühzeitig ein interdisziplinäres Konsil zwischen Kinderrheumatologen und orthopädischem Rheumatologen zu planen, um frühzeitig chirurgische Interventionen zu erörtern, die dann erforderlichenfalls rechtzeitig angegangen werden können. Die vorliegende Arbeit gibt eine Übersicht über die orthopädisch-rheumatologischen Indikationen und Möglichkeiten.

Juvenile Idiopathic Arthritis (JIA) patients living in areas with high prevalence of tick-borne-encephalitis-virus-(TBEV)-infection are recommended for administration of inactivated TBE-vaccination. However, there are serious concerns regarding protective vaccine-induced immune responses against TBEV in immunocompromised patients. The present study aimed to analyze the humoral and cellular immune response to TBE-vaccination in previously TBE-vaccinated JIA patients compared to healthy controls (HC) including investigation of IgG-anti-TBEV avidity, neutralization capacity, cellular reactivity by IFNgamma-ELISPOT and cytokine secretion assays. Similar IgG-anti-TBEV antibody concentrations, neutralization titers and cellular reactivity were found between JIA and HC. The number and the early timing of booster vaccinations after primary vaccination had the most prominent effect on neutralizing antibodies in JIA and on IgG-anti-TBEV concentrations in both JIA and HC. Administration of booster vaccinations made it more likely for JIA patients to have IgG-anti-TBEV concentrations ≥165 VIEU/ml and avidities >60%. TNF-alpha inhibitors had a positive and MTX administration a negative effect on humoral immune responses. In conclusion, irrespective of having JIA or not, vaccinated children showed similar humoral and cellular immunity against TBEV several years after primary TBE-vaccination. However, in JIA, booster vaccinations mounted a significantly higher humoral immune response than in JIA without boosters. Our results highlight the need for timely administration of boosters particularly in JIA. Although immunosuppressive treatment at vaccinations in diagnosed JIA had a negative effect mainly on TBEV-specific cellular immunity, most JIA patients mounted a favorable humoral immune response which was maintained over time. Thus, successful TBE-vaccination seems highly feasible in JIA patients with immunosuppressive regimens.

BackgroundThe ProKind Commission of the Society for Paediatric and Adolescent Rheumatology (GKJR) has developed evidence- and consensus-based protocols for the diagnosis and therapy of children and adolescents with defined rheumatic diseases (e.g., [1]). In the ProKind-Rheuma project, it is now investigated whether the protocols are followed in everyday clinical practice and what the treatment-associated outcomes are.ObjectivesTo investigate the mode of treatment and treatment response in patients with polyarticular juvenile idiopathic arthritis (pJIA).MethodsProKind-Rheuma is a multicenter prospective non-interventional observational study. Patients with pJIA enrolled until 17/1/2022 were included into this analysis. Treatments and outcomes up to the 3-month follow-up visit (3FU) were analyzed. Disease states were categorized based on the 2021 cJADAS10 cutoffs [2].ResultsTo date, 18 pediatric rheumatology facilities have participated in ProKind-Rheuma. Data from 203 patients with JIA are available. Of those, 44% have oligoarthritis, 36% polyarthritis, 9% systemic JIA, 6% enthesitis-related arthritis and 3% psoriatic arthritis.In total, 76 patients were diagnosed with pJIA, 38 with already completed 3FU:For 23 patients with pJIA and completed 3FU, we were able to analyze the protocol-defined [1] treatment goal of at least “minimal improvement”. In total, 18 (78%) achieved minimal improvement, 5 (22%) missed it. For 4 of those 5 patients, the underlying MTX therapy was escalated to a bDMARD (3 changed to MTX+bDMARD-combi, 1 to bDMARD-mono). In 3 other patients, therapy was also escalated to an MTX+bDMARD-combi.Between baseline and 3FU, 72% achieved cJADAS10-disease state improvement (Table 1) by at least one category (range 1 - 2), 0% decreased.Table 1.*based on non-missing valuesAt Baseline allAt Baseline with 3FUAt 3FUTotal7638Female, n (%)58 (76)30 (79)Age (years), Mdn (IQR)9 (3-12)7 (2-12)7.5 (3-12)Time since diagnosis (months), Mdn (IQR)0 (0-1)0 (0-1)4 (3-4)RF-positivity, n (%)8 (11)3 (8)Number of active joints (arthritis), Mdn (IQR)7 (4-12)7 (5-12)2 (0-4)JADAS10 (0-40), Mean (SD) (NBL+3FU= 23)18.6 (7.4)19.6 (7.6)7.2 (4.2)cJADAS10 (0-30), Mean (SD) (NBL+3FU= 29)16.3 (5.9)16.7 (6.1)7.1 (4.1)State of inactive disease (cJADAS10≤2.5), n (%*)0 (0)0 (0)4 (13)State of minimal disease activity (2.51 (2)1 (3)9 (28)State of moderate disease activity (533 (54)17 (50)18 (56)State of high disease activity (cJADAS10>16), n (%*)27 (44)16 (47)1 (3)CHAQ (0-3), Mean (SD)0.8 (0.8)0.9 (0.8)0.3 (0.5)Pain (NRS 0 - 10), Mean (SD)4.3 (3)4.7 (3)2.2 (2.7)PedsQL 4.0 total score, Mean (SD)66.3 (22.2)65.4 (21.8)78.4 (17.6)Intraarticular glucocorticoids > 4 joints (ever), n (%)12 (16)5 (13)7 (18)Glucocorticoid pulses (ever), n (%)22 (29)12 (32)13 (34)Methotrexate, n (%)56 (74)31 (82)34 (90)bDMARDs, n (%)7 (9)2 (5)9 (24)Within the first 3 months after diagnosis, the treatment pathways proposed by the ProKind Commission [1] were followed in about three-quarters of patients: i) 5 (13%) received MTX and intra-articular glucocorticoid injections in more than 4 joints (IAGC), but no high-dose intravenous glucocorticoid pulse (HDGC) or bDMARD; ii) 8 (21%) received MTX and HDGC (no bDMARD, no IAGC); iii) 16 (42%) patients received MTX, of whom 4 received a bDMARD up to or at the 3FU (no HDGC, no IAGC). Nine (24%) patients were not treated with MTX or did not fit any of these categories, mostly due to starting bDMARD therapy in conjunction with HDGC or IAGC.ConclusionIn the routine care of JIA patients with polyarthritis, the proposed treatment protocol and treat-to-target strategy are followed in most patients. At 3FU, improvements of JADAS10 and other outcomes were evident, with 41% having achieved inactive or minimal active disease.ProKind is funded by the Innovation Fund “Gemeinsamer Bundesausschuss”, FKZ: 01VSF18031References[1]Horneff et al. Pediatric Rheumatology 2017; 15:78[2]Trincianti et al. Arthritis Rheumatol. 2021 Nov; 73(11):1966-1975AcknowledgementsWe are grateful to all physicians, medical professionals and everyone else who has so far contributed and supported the ProKind-Rheuma project.Moreover, we want to express special gratitude to all patients and their parents for their participation.Disclosure of InterestsSascha Eulert: None declared, Kristina Vollbach: None declared, Klaus Tenbrock: None declared, Jens Klotsche: None declared, Dirk Foell Speakers bureau: Speaker fees/honoraria from Boehringer, Novartis, Werfen and Sobi, Grant/research support from: Novartis and Sobi, Johannes-Peter Haas: None declared, Frank Weller-Heinemann: None declared, Sonja Mrusek: None declared, Prasad Oommen: None declared, Daniel Windschall Speakers bureau: Research support and speakers fee: Pfizer, Novartis, Abbvie, Medac, Sobi, Canon, Grant/research support from: Research support and speakers fee: Pfizer, Novartis, Abbvie, Medac, Sobi, Canon, Kirsten Moenkemoeller: None declared, Tilmann Kallinich: None declared, Markus Hufnagel: None declared, Ivan Foeldvari Consultant of: Addvisory board: Hexal, Novartis, Pfizer, Toni Hospach Consultant of: Advisory board: Sobi, Novartis, Moritz Klaas: None declared, Michael Rühlmann: None declared, Ralf Trauzeddel: None declared, Normi Brueck: None declared, Catharina Schütz: None declared, J. B. Kuemmerle-Deschner: None declared, Ariane Klein: None declared, Kirsten Minden Speakers bureau: Speaker: Pfizer, Novartis, Gerd Horneff: None declared

BackgroundAlthough regular physical activity (PA) is known to have many health-related benefits for children and adolescents with chronic conditions, JIA has been associated with reduced physical activity (PA) and increased time spent sedentary [1]. Sedentary behaviour is an important consideration alongside PA when examining the contribution of both behaviours to the health of young patients. Because young people show more complex but less structured movement behaviour than adults, capturing their many spontaneous and impulsive movements is a great challenge for PA assessment.ObjectivesSince accurate, objective measures of PA related to potential factors promoting PA have been scarce, this study aimed i) to objectively assess moderate to vigorous PA (MVPA) as well as sedentary behaviour and ii) to identify facilitators for PA in patients with JIA.MethodsWithin the framework of the ActiMON study as part of the TARISMA research network, data were collected at several German paediatric rheumatology centres. Objectively assessed PA of adolescent patients with JIA was recorded exclusively during school months in the period June - December 2021 using the accelerometer ActiGraph wGT3X-BT laterally on the right hip. In accordance with the International Children’s Accelerometry Database criteria [2], ActiGraphs were worn on seven consecutive days during all waking hours, including at least 4 valid weekdays and 1 weekend day (wear time >8 hours) in the evaluation. Self-reported PA-related data were measured using a physical activity questionnaire (PAQ), while clinical parameters were used from the National Paediatric Rheumatologic Database (NPRD). Achievement of the WHO recommendation of at least 60 minutes MVPA per day and compliance with the national recommendation of no more than 120 minutes of daily screen time were determined among 12- to 18-year-olds.ResultsData of 41 patients (mean age 14.6 ± 1.9 years, female 73%, patients’ disease duration 7.9 ± 4.6 years, polyarthritis 40%, cJADAS-10 2.3 ± 2.4) were available for evaluation. Almost 71% of participating patients met the WHO-recommended minimum level of MVPA, while the average daily step count achieved was 7804. Almost 80% of the patients rated their motor and coordination skills as being good. The most frequently mentioned reasons for doing sports (multiple answers possible) included feeling good (78%), giving energy (70%), pleasure (65%) and enjoyment (60%). Patients whose parents are active in sports and support their child in finding a suitable offer were more likely to fulfil the WHO recommendation than patients without family support. The average daily time spent sedentary was 10.5 ± 1.9 hours. About half of the patients reported spending more than 4 hours a day with screen-based media, especially video games.ConclusionThese preliminary results suggest that while a large proportion of patients achieve the WHO recommended minimum level of PA, they exhibit very pronounced sedentary behaviour with increased screen use. In addition to controlled disease activity as a well-known prerequisite for achieving PA recommendations, these results also point to the importance of family support and emotional well-being. With ongoing recruitment, further potential facilitators and barriers to PA should be identified in more in-depth analyses.References[1]Gualano B et al. Physical activity for paediatric rheumatic diseases: standing up against old paradigms. Nat Rev Rheumatol 2017;13:368-379.[2]Sherar LB et al. International children’s accelerometry database (ICAD): design and methods. BMC Public Health 2011;11:485.AcknowledgementsActiMON is funded by the Federal Ministry of Education and Research (01EC1902F).Disclosure of InterestsFlorian Milatz: None declared, Ralf Trauzeddel: None declared, Tilmann Kallinich: None declared, Moritz Klaas: None declared, Hermann Girschick: None declared, Sandra Hansmann: None declared, Gerd Horneff: None declared, Daniel Windschall Speakers bureau: Pfizer, Novartis, Abbvie, Medac, Sobi, Canon, Johannes-Peter Haas: None declared, Nadja Baumeister: None declared, Martina Niewerth: None declared, Kirsten Minden Speakers bureau: Pfizer; Novartis

BackgroundIn recent years, transition clinics have been set up at an increasing number of paediatric rheumatology sites in Germany to reduce identified deficits in the care of young people with rheumatic diseases1. In addition, the German Rheumatic Diseases League (Deutsche Rheuma-Liga, DRL), the largest self-help organisation in Germany, has been offering support services for young people in transition since 2016, including the interactive website www.mein-rheuma-wird-erwachsen.de.ObjectivesTo assess the transition competence of young people with juvenile idiopathic arthritis (JIA) and their knowledge of self-help services.MethodsCross-sectional data of the National Paediatric Rheumatology Database (NPRD) from 2016 to 2020 were used to evaluate the health-related transition competence of young people with JIA aged ≥16 years. Health-related knowledge and health-care competence were assessed using a modified self-report instrument2 on a 4-point Likert scale as part of routine documentation in the NPRD. Young people were also asked about their information behaviour and knowledge of new support services. Linear mixed models were used to determine whether health-related transition competence changed between 2016 and 2020, adjusted for disease duration.ResultsDuring the years 2016 to 2020, between 1.908 to 2.536 patients with JIA aged ≥16 years were annually recorded in the NPRD from 56 to 61 paediatric rheumatology sites. The annual patient collectives comprised 34-39% oligoarthritis, 23-26% RF-negative or RF-positive polyarthritis and 22-27% enthesitis-related arthritis cases. In the years from 2016 to 2020, about one-third of patients had inactive disease (cJADAS-10≤1) and about 60% had no functional limitations (CHAQ=0).Over the years, the proportions of patients who rated their disease knowledge and health care competence as “very well” increased significantly in most areas. Although over time, no increase in numbers of patients seeking information about their disease outside of rheumatology consultations were recorded (2016: 22.8%; 2020: 20.9%), awareness of the DRL’s new website for young people with rheumatic diseases increased from 7.7% in 2016 to 26.9% in 2020. Compared to those who were unaware of the new website, those who knew about the website were more likely to have received care in rheumatology settings that offer transition clinics and were more likely to be girls (75% vs 65%), to attend high school (51% vs 46%) and to be slightly older (17.6 vs 17.1 years).ConclusionThe transition competence of young people with JIA seems to have improved over the last five years. During this time, more transition services were made available for young people with rheumatic diseases. However, most young people are not yet aware of these services. Moreover, the effectiveness of the different measures/interventions has yet to be evaluated.References[1]Luque Ramos A et al. Semin Arthritis Rheum 2017;47:269-75.[2]Herrmann-Garitz C et al. Gesundheitswesen 2017;79:491–6.Table 1.Health-related transition competence in JIA patients ≥16 years who participated in the NPRD201620182020p (difference over time)PatientsN=2536N=2068N=1908Disease duration, years6.7±4.97.2±5.07.6±5.1DMARDs at documentation, %576263Disease-related knowledge (best answer “very well”), %N=1992N=1598N=1265name of illness3542420.001names of medicines5459560.717what medicines are for5054520.357who to contact in case of health problems5965650.015influence of smoking, drugs, and alcohol on disease4955540.002how to make a doctor’s appointment6868650.087which doctors are responsible after leaving paediatric care4246490.031Health-care competence (best answer “most of the time”), %N=1784N=1443N=1143inform my doctor of any unusual changes in my health6672690.038keep information about my illness8184840.281ask my own questions5156550.016answer the questions I am asked6973740.014take care of my health concerns and needs6671690.041attend the consultation alone5961610.599speak up for myself and say what I need6468680.537AcknowledgementsThe NPRD has been funded by the Federal Ministry of Health and the companies Abbvie, Chugai, ask, Novartis, PfizerDisclosure of InterestsKirsten Minden Speakers bureau: Pfizer, Novartis, Consultant of: Pfizer, Novartis, Martina Niewerth: None declared, Susanne Schalm: None declared, Ivan Foeldvari: None declared, Johannes-Peter Haas: None declared, Gerd Horneff: None declared, Daniel Windschall Speakers bureau: Pfizer, Novartis, Abbvie, Medac, Sobi, Canon, Grant/research support from: Pfizer, Novartis, Abbvie, Medac, Sobi, Canon, Tilmann Kallinich: None declared, Frank Dressler: None declared, Frank Weller-Heinemann: None declared, Rainer Berendes: None declared, Toni Hospach Consultant of: SOBI, Novartis, Markus Hufnagel: None declared, Maria Haller: None declared, Sandra Hansmann: None declared, Jens Klotsche: None declared

BackgroundUveitis is an extra-articular manifestation of Juvenile idiopathic arthritis (JIA) with a prevalence of up to 20% developing most frequently in young girls and patients positive for antinuclear antibodies (ANA). Untreated and uncontrolled uveitis may lead to vision-threatening complications and even blindness.ObjectivesThe main objectives of the analyses were to determine the visual prognosis, uveitis complications and necessity of ocular surgery during the first five years of ocular disease. The likelihood of achieving an inflammation-free phase or even a remission without medication were investigated.MethodsThe Inception Cohort of Newly diagnosed patients with JIA (ICON) was initiated in 2010 in order to prospectively follow JIA patients up to 10 years after JIA disease onset. 953 Patients were assessed at enrollment, three-monthly during the first year, and six-monthly afterwards by a standardized physician’s and patient’s case report form including clinical parameters, treatment data and several laboratory parameters such as ESR, CRP or S100A12. Patients who developed uveitis underwent a regular ophthalmological assessment. The treating ophthalmologist three-monthly completed an additional questionnaire, documenting the anterior chamber (AC) cell grade, current uveitis activity (UA) and UA during the previous three months, best corrected visual acuity (BCVA), uveitis-related complications, previous ocular surgery, current topical treatment and clinical course of uveitis and additional parameters. Inactive uveitis was defined by AC cell grade of 0, quiescence of uveitis by inactive uveitis for at least 6 months, and remission by inactive uveitis for at least 6 months without topical steroids or systemic anti-inflammatory medication (steroids or DMARDs).ResultsA total of 133 children developed uveitis in the JIA disease course, of which 97 patients were documented via the ophthalmological questionnaire for at least two years resulting in a mean follow-up of 5.8 years (SD 1.8). 76% were female, 86% ANA positive, 70% oligoarthritis, and 22% rheumatoid factor negative polyarthritis and mean age at JIA onset was 3.1 (SD 2.1) and uveitis onset at 4.4 (SD 2.2) years. The mean duration between JIA onset and uveitis onset was 15.7 (SD 15.6) months. At least one ocular complication was reported for 24% of patients at first uveitis documentation and 47% of patients had at least one ocular complication until the five year follow-up. Among those, posterior synechiae (31%) and cataract (27%) were the most frequent, followed by an increased IOP (12%) with or without glaucomatous changes. Ocular surgery was rarely necessary, and visual acuity remained quite good in the majority of patients: After five years, >90% had BCVA of ConclusionThe rate of ocular complications is already remarkable at uveitis diagnosis, and increases during uveitis disease course despite anti-inflammatory treatment. However, the visual acuity frequently remains unaffected, and the majority of patients achieve uveitis quiescence and even 40% uveitis remission within 5 years of follow-up.AcknowledgementsThe ICON study is funded by a research grant of the Federal ministry of education and research (BMBF, FKZ 01ER0812, FKZ 01ER1504A-C)Disclosure of InterestsJens Klotsche: None declared, Claudia Sengler: None declared, Frank Dressler: None declared, Dirk Foell: None declared, Ivan Foeldvari: None declared, Johannes-Peter Haas: None declared, Gerd Horneff Speakers bureau: Pfizer, Novartis, Janssen, Chugai, Abbvie, Grant/research support from: Pfizer, Novartis, MSD, Chugai, Roche, Abbvie, Toni Hospach Consultant of: SOBI, Novartis, Tilmann Kallinich: None declared, Ina Liedmann: None declared, Kirsten Moenkemoeller: None declared, Martina Niewerth: None declared, Frank Weller-Heinemann: None declared, Daniel Windschall: None declared, Arnd Heiligenhaus: None declared, Kirsten Minden: None declared, Karoline Baquet-Walscheid: None declared

Rheumatic diseases, such as juvenile idiopathic arthritis (JIA), are typically associated with acute pain mainly caused by inflammation. Chronic pain is described as pain lasting at least 3 months. In JIA patients chronic pain may occur despite successful treatment. Chronic pain and pain disorders frequently occur during the course of the disease despite successful control of inflammation.Possible interrelations between JIA and pain disorders are presented.Besides a review of the available literature, a retrospective cohort study was conducted, including 906 patients with a chronic pain disorder with somatic and psychological factors (CPD) and/or a complex regional pain syndrome type I (CRPS I). The frequency of pre-existing rheumatic illnesses was analyzed.The JIA is a risk factor for the development of a CPD. Especially polyarticular, extended oligoarticular, enthesitis-associated JIA and psoriatic arthropathy were found to be significantly associated with an increased risk for developing CPD. In contrast, an increased risk for development of CRPS I was not observed.Our study demonstrates JIA to be a risk factor for the development of chronic pain not only as a result from malpositioning or arthrosis but also as a chronic pain disorder (CPD). Further studies are necessary to clarify the relevance of disease activity and duration and also of psychological factors for the pathogenesis.HINTERGRUND: Bei rheumatischen Erkrankungen wie der juvenilen idiopathischen Arthritis (JIA) tritt klassischerweise ein entzündungsbedingter Akutschmerz auf. Von chronischen Schmerzen spricht man ab einem Zeitraum von 3 Monaten. Schmerzen können sich trotz erfolgreicher Therapie als Schmerzerkrankung zu einem eigenständigen Krankheitsbild entwickeln. Chronische Schmerzen und Schmerzerkrankungen können trotz guter Kontrolle der entzündlichen Aktivität auch im Verlauf einer JIA entstehen.Es erfolgt die Darstellung möglicher Zusammenhänge zwischen einer JIA und Schmerzerkrankungen.Neben einem Review der vorhandenen Literatur wurde eine retrospektive Kohortenstudie mit 906 Patienten mit chronischer Schmerzstörung mit somatischen und psychischen Faktoren (CSSPF) und/oder einem komplexen regionalen Schmerzsyndrom Typ I (CRPS I) durchgeführt und die Häufigkeit rheumatologischer Vorerkrankungen untersucht.Die JIA ist ein Risikofaktor für die Entwicklung einer CSSPF. Vor allem die polyartikuläre, die „extended“ oligoartikuläre JIA, die Enthesitis-assoziierte JIA und die Psoriasisarthritis haben ein signifikant erhöhtes Risiko. Dagegen finden sich die persistierende oligoartikuläre und die systemische JIA nicht gehäuft in der Vorgeschichte von Patienten mit einer CSSPF. Im Gegensatz dazu wurde kein nennenswert erhöhtes Risiko für die Entwicklung eines CRPS I beobachtet.Unsere Untersuchung zeigt, dass das Vorliegen einer JIA einen Risikofaktor nicht nur für die Entstehung chronischer durch Fehlstellungen bzw. Arthrose bedingter Schmerzen, sondern auch einer chronischen Schmerzstörung darstellt. Weitere Untersuchungen sind notwendig, um zu klären, welche Relevanz Krankheitsaktivität und -dauer, aber auch psychologische Faktoren auf die Entstehung haben.

Inflammatory rheumatic diseases in childhood and adolescence are a special challenge in the treatment of acute trauma. The pharmaceutical treatment strategies for children and adolescents have been modified.Which special aspects must be considered in young patients suffering from rheumatism when a trauma necessitates an operative procedure?A literature search was carried out to elaborate recommendations for the practice.The joint-related alterations in young patients suffering from rheumatism differ with respect to the differently altered inflammatory rheumatic destruction. The extent of these inflammatory destructive alterations dictates the operative approach. Consequences arise in paying attention to the concurrent medication with respect to avoidance of events triggering an exacerbation and tissue infections. The bone strength necessitates an individualized selection of implants and sometimes influences the duration of follow-up treatment. In the early stages of the inflammatory process the approach in cases of trauma is no different to that for healthy patients but in later stages (Larsen stage III) it does differ.An interdisciplinary concept can help to avoid disadvantages in the treatment of the underlying disease. Due to the special dysplastic anatomy and tissue alterations, trauma in these patients is a particular challenge.

Adalimumab (ADA) has become a valuable treatment option for juvenile idiopathic arthritis (JIA). The importance of combination with methotrexate (MTX) is unclear.Data from the German Biologics in Paediatric Rheumatology (BIKER) registry are reported. Response to treatment was analysed using JIA American College of Rheumatology (ACR) scores, 10-joint Juvenile Arthritis Disease Activity Score (JADAS10), and improvement of functional status and ACR inactive disease criteria. Compa-risons between rates of adverse events (AEs) and serious adverse events (SAEs) provided data for the safety assessment.Overall, 584 patients with non-systemic JIA started ADA therapy, 61% of whom received concomitant MTX treatment at baseline. The latter patients were younger (p 0.001), with shorter disease duration (p = 0.001), more frequently had antinuclear antibodies (p = 0.04), and had higher baseline JADAS10 scores (p = 0.03). In patients with ADA monotherapy, enthesitis-related arthritis (p = 0.004) and presence of human leucocyte antigen-B27 (p = 0.008) were documented more often. Mean treatment duration in both cohorts was 15 months. Comparable last follow-up rates for JIA ACR 30/50/70/90% response, JADAS minimal disease activity, JADAS remission, and ACR inactive disease were, respectively, 75/72/64/49%, 66%, 46%, and 58% for ADA monotherapy, and 77/72/61/45%, 64%, 48%, and 55%, for ADA + MTX. During 1082 patient-years (PY) of ADA exposure, 725 AEs (67/100 PY), including 57 SAEs (5.3/100 PY), were reported. Serious infections were reported in 10 patients (0.9/100 PY) and 11 (1.0/100 PY) had varicella infections/zoster reactivation. Rates of AEs, SAEs, infectious events, and serious infections did not differ between the cohorts. Elevated transaminases (p = 0.005) and gastrointestinal events (p 0.0001) were reported more often in the combination cohort. Two pregnancies and no deaths were reported.ADA demonstrated an acceptable risk profile and high percentages of patients in both cohorts showed sufficient treatment response. No differences in treatment response or adherence to treatment were found.

ZusammenfassungProgressive Verlaufsformen der JIA, die durch medikamentöse Behandlung wenig beeinflussbar sind, münden nicht selten in eine endoprothetische Versorgung vor dem fünften Lebensjahrzent. Durch den Beginn im Kindesoder Jugendalter werden Wachstum und physiologische Gelenkentwicklung verändert. Daraus resultierende Bewegungseinschränkungen und Gelenkfehlstellungen beeinflussen Implantatwahl und Weichteilbehand-lung. Schmerzlinderung und Verbesserung der Lebensqualität nach endoprothetischer Versorgung sind beeindruckend. Dennoch ist die Lebensplanung mit Endoprothesen unterschiedlich zu prognostizieren und abhängig vom Implantationsort.

Background:Juvenile idiopathic arthritis (JIA) associated uveitis is an extra-articular manifestation of the JIA disease that may cause vision-threatening complications and an uncontrolled uveitis may even lead to blindness. Uveitis occurs in up to 20% of patients with JIA, depending on the JIA category. The majority of patients develop uveitis within the first two years after JIA symptom onset, but uveitis can continue into adulthood.Objectives:The main objective of this study was to analyze the risk for uveitis events after discontinuing disease-modifying antirheumatic drugs (DMARD) in patients with extended oligoarthritis and rheumatoid factor (RF)-negative polyarthritis.Methods:Data of the two ongoing multicenter biologic registers: German Biologics in Pediatric Rheumatology (BiKeR) and the Juvenile arthritis Methotrexate/Biologics long-term Observation (JuMBO) were used to analyze the adverse-event (AE) and events of special interest (ESI) reports about uveitis events during treatment and after discontinuation of DMARDs. Biker started recruitment of children and adolescent patients with JIA exposed to biological (b) or conventional (cs) DMARD’s in 2001. The patients were further followed in JuMBO after reaching the age of 18 or transitioning to an adult rheumatologist. Disease characteristics, treatment data, AE’s and ESI’s were reported by the pediatric or adults rheumatologist, respectively.Results:A total of 2,041 patients with RF-negative polyarthritis (n=1,280) or extended oligoarthritis (n=761) were included into the analyses. The mean follow-up of this study was 7.6 years (SD 5.3). About half of the patients were enrolled in BiKeR with start of etanercept (1,137, 55.7%), followed by 635 (31.1%) patients with start of methotrexate (MTX) monotherapy or adalimumab (ADA, n=198, 9.7%). A history of uveitis was reported for 238 (11.7%) patients at enrolment in BiKeR. More patients with a history of uveitis treated with ADA were included in BiKeR initiating ADA (n=98 of 238, 41.2%). Patients with uveitis had a lower age at JIA onset in comparison to patients without uveitis (mean 3.6 (SD 3.0) versus 7.0 (SD 4.5) years). A total of 142 recurrent (84% of 169) uveitis events were reported in 93 patients and for 27 patients (1.3% of 2,041) was an incident uveitis reported during follow-up. More than one uveitis event was reported for 32 patients with a maximum number of 4 uveitis flares in 3 patients. Nineteen uveitis flares (11.2% of 169) were reported for patients after the age of 18. The longer the time since DMARD discontinuation the fewer uveitis events occurred. Uveitis events were significantly more often reported in the first 24 months after MTX discontinuation (2 at last MTX intake had a higher likelihood for uveitis events (OR=1.40, 95%CI: 1.02 to 1.92).Conclusion:This is the first study that analyzed the risk of uveitis after DMARD withdrawal. Uveitis relapses are common. Patients who discontinued DMARD therapy were at high risk for uveitis within the first 3 to 24 months after discontinuation. Rheumatologists and ophthalmologists should be aware about this risk which should lead to a regular uveitis screening after DMARD withdrawal.Disclosure of Interests:Jens Klotsche: None declared, Ariane Klein: None declared, Martina Niewerth: None declared, Tilmann Kallinich: None declared, Daniel Windschall: None declared, Johannes-Peter Haas: None declared, Frank Weller-Heinemann Speakers bureau: Pfizer, Abbvie, SOBI, Roche, Novartis, Toni Hospach: None declared, Frank Dressler: None declared, Kirsten Minden: None declared, Gerd Horneff: None declared

Background:Various treatment strategies are used for children with newly diagnosed polyarticular JIA. MTX is usually prescribed, sometimes in combination with high-dose intravenous glucocorticoid pulses (HDGC) or multiple intra-articular GC injections (IAGC). These different approaches were considered in the German consensus-based treatment protocols for polyarticular JIA1, they were also the leading therapies in patients with rheumatoid factor-negative polyarthritis (RF- PA) included in the JIA inception cohort ICON.Objectives:To compare the effectiveness of three different treatment strategies in nearly DMARD-naïve patients with RF- PA.Methods:Patients with RF- PA who were included in the ICON cohort and received one of the following treatments within the first three months were considered for the analysis: Group 1: MTX + IAGC in >4 joints, Group 2: MTX + HDGC, Group 3: MTX, no IAGC in >4 joints, no HDGC. Propensity score-adjusted group differences in outcomes after one and two years were analysed by linear and logistic regression analyses.Results:The analysis included data from 150 patients (79% female, mean age 6.7±4.8 years) enrolled in ICON 1.6±1.9 months after the diagnosis of RF- PA, of whom 52 were in Group 1, 54 in Group 2 and 44 in Group 3. Disease activity did not differ significantly between the groups at treatment start (cJADAS-10 16.7±4.7, 15.8±5.7, 15.9±6.5, respectively).Of the total group, at 1- and 2-year follow-up (FU), 60.9%/60.1% and 52.3%/58.8% of patients had inactive disease (cJADAS ≤1/Wallace criteria2), 21.3% and 35.6% were in remission off drug2, and mean cJADAS-10 scores were 2.6±3.9 and 3.0±3.5, respectively. 60.5% and 67.0% had no functional limitations (CHAQ=0).Patients in Group 1 more often had an inactive disease (according to Wallace2) at the 1-year FU and tended to have inactive disease more often at 2-year FU than patients in Group 3 (78.1% vs. 45.2%, p=0.025; 73.3 vs. 49.1%, p=0.075, respectively). Group 2 patients (inactive disease in 56.1% and 53.4% at 1- and 2-year FU) did not differ significantly from either Group 1 or Group 3. In addition, Group 1 patients had a significantly better quality of life than patients of Group 2 at the 2-year FU (mean PedsQL 4.0 total score 90.4±9.3 vs. 83.8±11.2, p=0.031). At that time, Group 3 patients had a mean PedsQL 4.0 total score of 85.0±14.6, which was not significantly different from either Group 1 or 2.On the other hand, Group 1 patients tended to develop new uveitis more frequently within the first two years of treatment than patients in Groups 2 and 3 (13% vs. 2.2% and 3.6%, p=0.101 and 0.131, respectively). At the 2-year FU, patients in Group 1 also had a significantly lower mean height SDS than patients of Group 3 (-0.3±1.1 vs. 0.2±1.1, p=0.038). Mean height SDS was lowest (-0.5±0.8) in patients in Group 2 and significantly lower than in Group 3 (0.019). Mean body mass index SDS also differed significantly between the groups at 2-year follow-up. The mean BMI SDS was highest in Group 1 patients (0.2±0.8), differing significantly from Group 2 (-0.3±0.7, p=0.014) and Group 3 (-0.4±1.1, p=0.023).There were no significant differences in inactive disease (according to cJADAS) and functional status (CHAQ) between the three groups at 1- and 2-year FU. Over time, treatments were very different in the three groups. In Group 3, biologics were used significantly more often over time than in group 1 (54.0% vs. 18.3%, p=0.014), and Group 2 patients received bDMARDs in 36.1%.Conclusion:While patients with numerous early joint injections seem to achieve inactive disease more frequently and earlier, they have a slightly smaller body height and tend to develop uveitis slightly more often than patients with more intensive DMARD therapy. However, the differences are small between the groups. Further comparative effectiveness studies with higher patient numbers are needed to identify particularly effective and safe treatment strategies.References:[1]Horneff et al. Pediatric Rheumatology 2017;15:78.[2]Wallace et al. Arthritis Care Res (Hoboken) 2011;63:929-36.Acknowledgements:The ICON study is funded by a research grant of the Federal ministry of education and research (BMBF, FKZ 01ER0812, FKZ 01ER1504A-C).Disclosure of Interests:Kirsten Minden Speakers bureau: Pfizer, Abbvie, Consultant of: Novartis, Tobias Schwarz: None declared, Frank Dressler: None declared, Ivan Foeldvari Consultant of: Gilead, Novartis, Pfizer, Hexal, BMS, Sanofi, MEDAC, Johannes-Peter Haas: None declared, Gerd Horneff Speakers bureau: Pfizer, Consultant of: Novartis, Toni Hospach Consultant of: Novartis, Jasmin Kümmerle-Deschner: None declared, Kirsten Moenkemoeller: None declared, Frank Weller-Heinemann Speakers bureau: Pfizer, AbbVie, SOBI, Roche, Novartis, Klaus Tenbrock: None declared, Martina Niewerth: None declared, Claudia Sengler: None declared, Dirk Foell: None declared, Jens Klotsche: None declared

Background:Although children and adolescents are less likely to develop COVID-19 and generally show milder disease courses, it is unclear what impact the SARS-CoV2 infection has on children and adolescents with rheumatic and musculoskeletal disease (RMD). Due to their underlying disease as well as therapeutic immunosuppression these patients may be at higher risk of being more severely affected by SARS-CoV2. Furthermore, SARS-CoV2 infection might trigger a flare of the underlying disease.Objectives:To evaluate clinical characteristics and disease course of COVID-19 in children and adolescents with RMD and to analyze possible effects of SARS-CoV2 infection on the underlying disease under different therapeutic regimens.Methods:Data from juvenile patients with RMD recorded via the SARS-CoV2 questionnaire within the National Pediatric Rheumatology Database and the registry for hospitalized children and adolescents with COVID-19 of the German Society for Pediatric Infectious Diseases were analyzed. In addition to age, sex and diagnosis, information was collected about the date and method of a positive SARS-CoV2 testing, reason for testing, on clinical manifestations, disease course, treatment and outcome of COVID-19, on drug therapy at the time of virus detection, on disease activity (NRS 0 – 10, 0 = best) of the underlying disease at the last visit before and after the SARS-CoV2 infection.Results:From April 17th 2020 until January 25th 2021, data of 67 patients with RMD and confirmed SARS-CoV2 infection were collected. Mean age was 13.5 ± 3.9 years with equal sex distribution. The majority of patients were diagnosed with juvenile idiopathic arthritis (JIA, 64%), 12 (18%) patients had an autoinflammatory disease (FMF, CAPS, PFAPA, TRAPS) and 5 (7%) a connective tissue disease. Fifty-two patients (78%) were treated with a disease modifying antirheumatic drug (DMARD), 39% with a biological DMARD and 9% systemic glucocorticoids at the time of SARS-CoV-2 infection. Nineteen patients (28%) were tested for SARS-CoV-2 because of typical symptoms, the majority (67%) because of contact to an infected person. PCR was used most often (in 60 %).52 patients (78%) developed symptoms of COVID-19, 15 patients remained asymptomatic. The most common symptom of COVID-19 was rhinitis (42%) and fever (38%), followed by fatigue (34%), taste/smell disorder (33%), sore throat (27%) and cough (23%).Disease severity was graded as mild in 44 of 52 (85%) symptomatic patients, only two patients were hospitalized, one of whom required intensive care and died of cardiorespiratory failure 3 days after symptom onset. In 22 of 26 (85%) SARS-CoV2-positive patients, no relevant increase in disease activity (difference in NRS ≤ 1 before/after infection) of the underlying disease was observed 31 days after symptom onset (median, IQR 17-52 days). One patient, who had paused tocilizumab for 2 doses, experienced a flare of his seronegative polyarthritis 2 months after asymptomatic SARS-CoV-2 infection.Conclusion:In our cohort, the clinical picture of COVID-19 in children and adolescents with RMD was similar to that of healthy peers. The majority of patients showed mild disease course with good outcome under various medications, however, one patient with a severe course of COVID-19 died. In addition, SARS-CoV2 infection does not appear to have a relevant impact on the underlying disease activity, whereas discontinuation of therapy might pose a risk of flare.Disclosure of Interests:None declared.

Background:In patients with JIA, growth impairment and variance in body composition are well-known long-term complications that may be associated with prolonged drug therapy (e.g. glucocorticoids) as well as impaired physical and psychosocial well-being. An increased accumulation of body fat represents a significant risk factor for metabolic abnormalities and a modifiable variable for a number of comorbidities. Recently, evidence has emerged in favour of the potential negative influence of overweight on the course of the disease and treatment response [1].Objectives:The study aimed a) to estimate the prevalence of underweight, overweight and obesity in children and adolescents with JIA compared to the general population, and b) to investigate correlates of patients’ weight status.Methods:A cross-sectional analysis of physicians’ recorded body weights and heights of patients with JIA enrolled in the NPRD in the year 2019 was performed. Underweight (BMI 90th) and obesity (BMI >97th) were defined according to age- and sex-specific percentiles used in the German reference system. For comparison with data from the general population [2], sex- and age-matched pairs of 3-17-year-old patients and controls were generated. A multinomial logistic regression analysis was performed to examine the association between weight status and patients’ clinical and self-reported outcomes.Results:In total, data from 6.515 children and adolescents with JIA (age 11.2 ± 4.1 years, disease duration 4.9 ± 3.8 years, 67% girls, 40% persistent oligoarthritis) were included. Of these, 3.334 (age 5.9 ± 2.1 years, 52.5% girls) could be considered for matched-pair analysis. Compared with the general population, patients underweight, overweight and obesity rates were 10.6% (vs. 8.1%), 8.8% (vs. 8.5%) and 6.1% (vs. 5.7%), respectively. No significant sex differences were found in either group. Largest difference in prevalence was registered for underweight, specifically in the age group 3-6 years (12.9% patients vs. 5.9% controls). Similar to the general population, higher rates of overweight were observed in adolescent patients than in affected children (19.1% age group 11-13 vs. 8.4% age group 3-6). While the highest underweight prevalence was registered in patients with RF+ polyarthritis (16%), patients with Enthesitis-related arthritis (22%), psoriatic arthritis (21%) and systemic JIA (20%) showed the highest overweight rates (including obesity). Younger age (OR = 0.51, 95% CI = 0.31-0.83), more frequent physical activity (OR = 0.92, 95% CI = 0.85-0.99) and high parental vocational education (OR = 0.39, 95% CI = 0.18-0.80) were independently associated with a lower likelihood of being overweight/obese.Conclusion:The overall prevalence of underweight, overweight and obesity in children and adolescents with JIA is comparable to that found in the general population. Behavioural health promotion, including regular physical activity, as part of the treatment strategy in JIA should preventively already begin at preschool age and necessarily be made accessible to patients of all educational levels.References:[1]Giani T et al. The influence of overweight and obesity on treatment response in juvenile idiopathic arthritis. Front Pharmacol 2019;10:637.[2]Schienkiewitz A et al. BMI among children and adolescents: prevalences and distribution considering underweight and extreme obesity. Bundesgesundheitsbl 2019;62:1225–1234.Acknowledgements:The National Paediatric Rheumatological Database has been funded by AbbVie, Chugai, Novartis and GSK.Disclosure of Interests:Florian Milatz: None declared, Jens Klotsche: None declared, Martina Niewerth: None declared, Jana Hörstermann: None declared, Daniel Windschall: None declared, Frank Weller-Heinemann Speakers bureau: Pfizer, AbbVie, SOBI, Roche and Novartis., Frank Dressler: None declared, Rainer Berendes: None declared, Johannes-Peter Haas: None declared, Gerd Horneff: None declared, Kirsten Minden Speakers bureau: Pfizer, AbbVie, Consultant of: Novartis

ZusammenfassungHäufig werden Nachhol-, Auffrisch- und Indikationsimpfungen bei Patienten mit Autoimmunerkrankungen insbesondere unter Immunsuppression in Sorge vor Schüben der Grunderkrankung oder aufgrund von Unwissenheit über die meist gute Impfantwort über Jahre hinweg verschoben. Zum Zeitpunkt der Transition in die Erwachsenenrheumatologie besteht die Gelegenheit, die Vorgeschichte des Patienten aufzuarbeiten und den aktuellen Impfstatus zu überprüfen. Mögliche neue Motivation für Impfungen in diesem Lebensabschnitt stellen geplante Auslandsaufenthalte, eine Ausbildung im Gesundheitssystem oder ein Kinderwunsch dar. Um die Akzeptanz für Impfungen zu erhöhen, empfiehlt sich eine individualisierte Impfberatung unter Berücksichtigung des persönlichen Risikos einer schwer verlaufenden Infektion, die Messung von Ausgangsimpftitern, um unnötige Boosterungen zu vermeiden und – im Falle von Lebendimpfungen – der Einsatz von Impfstrategien, die ermöglichen, das Risiko für einen Schub der Grunderkrankung bzw. eine Erkrankung durch den Impfstamm zu minimieren. Aus diesem Grund werden hier immunologisch basierte Algorithmen als Diskussionsgrundlage vorgeschlagen, damit auch Patienten mit seltenen Erkrankungen und/oder immunsuppressiver Therapie nicht durch das Raster der Impfempfehlungen fallen.

Hintergrund: Bei verschiedenen chromosomalen Storungen wird ein erhohtes Risiko fur eine Juvenile Idiopathische Arthritis (JIA) vermutet. Vor allem bei Patienten mit einem Morbus Down (Trisomie 21) und einer JIA besteht daruber hinaus eine Unsicherheit bezuglich der geeigneten Therapie mit DMARDs, weil auch das Risiko bezuglich einer AML erhoht ist. Methode: In einer retrospektiven Fall-Kohorte eigener Patienten wurde das typische Verteilungsmuster und die Entzundungsaktivitat bei Patienten mit Trisomie 21 und JIA (T21-JIA) sowie deren Ansprechen und Nebenwirkungen von MTX im Vergleich zu JIA-Patienten mit seronegativer polyartikularer JIA (SNP-JIA) untersucht. Ergebnisse: Die elf eingeschlossenen T21-JIA Patienten (6 weibl./5 mannl.) hatten bei Diagnosestellung JIA ein mittleres Alter von 6 Jahren (1–13 Jahre). 9 T21-JIA Patienten (82%) hatten einen polyartikularen Verlauf und wurden mit MTX behandelt. T21-JIA Patienten hatten bei Diagnosestellung eine signifikant hohere Anzahl aktiver und schmerzhafter Gelenke (T-21 JIA 19,9 (± 10,9) vs. SNP-JIA 15,5 (± 6,5)) und prasentierten sich in 7 von 10 (70%) Fallen als SNP-JIA (gemas ILAR-Klassifikation, erwartet: 13–15%). Ein T21-JIA Patient hatte eine systemische, einer eine persistierende- und 2 eine extended oligoartikulare JIA. Im Verlauf entwickelten 81% der T21-JIA Patienten Gelenkskontrakturen. Kein T21-JIA Patient hatte im Verlauf eine Uveitis. Das Ansprechen auf MTX und die Frequenz von unerwunschten Arzneimittelwirkungen (UAW) war bei T21-JIA Patienten mit denen einer gematchten Vergleichstichprobe vergleichbar. Schlussfolgerung: T21-JIA Patienten zeigen gehauft einen polyartikularen Verlauf mit einer hoheren Anzahl betroffener Gelenke und ausgepragteren Bewegungseinschrankungen im Erkrankungsverlauf. Das Ansprechen und die UAW einer MTX-Therapie bei Patienten mit T21-JIA entspricht dem von JIA-Patienten mit einem normalen Karyotyp. Auch bei anderen chromosomalen Aberrationen namentlich Turner- (Monosomie XO), Klinefelter- (Poly-X, XXY) und Di George Syndrom (del 22q11.2) wird ein erhohtes Risiko fur das Auftreten einer JIA vermutet.

Myotone Muskeldystrophie (DM1) mit Phanotyp eines APS (autoimmune polyendocrine syndrome)

Zunehmend haufig werden Kinder und Jugendliche mit lokalisierten oder generalisierten muskuloskelettalen Schmerzen (kinder-)rheumatologisch vorgestellt, ohne dass eine zugrunde liegende rheumatologische Erkrankung diagnostiziert werden kann. Differenzialdiagnostisch mussen Schmerzerkrankungen bedacht werden. Eine lokalisierte chronische Schmerzsymptomatik ist seltener zu finden als die generalisierte Form. Vor allem das komplexe regionale Schmerzsyndrom (complex regional pain syndrome, CRPS) weist bei Kindern und Jugendlichen im Vergleich zum Erwachsenenalter einige Besonderheiten, nicht zuletzt bezuglich Behandlung und Prognose auf. Multimodale Therapieprogramme haben gute Erfolgsaussichten, die Therapie sollte zeitnah durch padiatrisch und schmerztherapeutisch erfahrene Behandler erfolgen, um eine Chronifizierung zu vermeiden.

The treatment of children and adolescents with juvenile idiopathic arthritis (JIA) has progressed substantially during recent years. Multiple different factors have played a role in this advancement: improved medical treatment due to the introduction of new drugs, structural improvements with provision of units specializing in childhood rheumatology, multidisciplinary treatment concepts, structured educational programs for patients and parents, improved functional treatment including sports therapy, and selective surgical and orthopedic interventions improving functional capacities. Current treatment strategies in JIA are aimed at achieving disease remission, i.e., control of disease activity and re-establishment of age-appropriate functional capacities. This review summarizes important developments in the conservative treatment of JIA. Part 2 deals with orthopedic and surgical treatment strategies.

In most cases of juvenile idiopathic arthritis (JIA), disease remission can be achieved by a multidisciplinary approach using modern medication. However, JIA is currently incurable An interdisciplinary concept is therefore needed for long-term development of affected joints and tendons. Only early interdisciplinary treatment strategies can improve long-term outcomes in patients with a complicated disease course or persistent disease activity, thereby delaying or even avoiding joint replacement. An early interdisciplinary assessment and treatment planning according to surgical orthopedic rheumatology aspects is now state of the art and will continue to be in the future, especially in treatment-refractory disease courses.

Taking part in physical education is an important element of social participation for children with chronic diseases. Nevertheless, children suffering from rheumatism mostly receive recommendations to stop sport activities either completely or partially, without underlying scientific guidelines.The aim was the development of an IT-tool based on scientific data in order to create individualized recommendations for sport activities plus verification of its practical feasibility.An interdisciplinary group of experts developed and approved a prototype of the rheumatism and sports compass (Rheuma und Sport Kompass, RSK) based on the literature and own experience. They considered individual health factors and biomechanics of sports functions. The prototype was tested, revised and reconsidered in an interim evaluation. The resulting RSKv1 was evaluated in a clinical observation phase with 61 patients. The results were subsequently incorporated into the final version of RSK during an interdisciplinary decision-making process. This was verified in a feasibility study with a follow-up survey of rheumatic patients with a RSK partial participation certification for physical education including: clinical assessment during 8 lessons of physical education and after 8 lessons of physical education. Teachers rated the RSK online after 8 lessons. The evaluation was descriptive and differences in mean values were tested.In this study 50 patients and 31 teachers were evaluated. The affliction of pain decreased in terms of frequency, amount and duration after physical education with RSK. No worsening in health was reported after participation in sports. The teachers rated the RSK as understandable, practicable and they felt confident to allow the patients to participate in classes. The RSK was rated significantly better than a standard certification text. With the RSK, patients can be advised to safely take part in physical education.

Background Vitamin D has been shown to have immunomodulatory properties in addition to its well-established role in the maintenance of mineral homeostasis and bone health. Conflicting data have been available regarding vitamin D status in children and adolescents with juvenile idiopathic arthritis (JIA) and its influence on the disease. Objectives To determine 25–0 hour-vitamin D3 (25-OHD) levels in patients with JIA, and to evaluate whether 25-OHD levels are associated with disease activity and disease course. Methods Serum 25-OHD levels were determined in a pair of serum samples from patients with JIA who were enrolled in the JIA inception cohort study ICON and prospectively followed for three years. The frequencies of 25-OHD deficiency (defined as Results In 360 patients with early JIA (48% OA, 27% rheumatoid-factor negative polyarthritis), 25-OHD levels were determined twice: after a median disease duration of 6.6 and of 13.0 months. The mean 25-OHD level of all JIA samples was insufficient (22.1 ng/ml, SD 7.8), but significantly higher than that of the control population (18.4 ng/ml, SD 10, p An insufficient mean 25-OHD level was found in 40% of JIA patients and in 25% in both samples each, sufficient levels in 13% and 7%, respectively. There were no significant differences in 25-OHD levels among the JIA categories. Disease activity, measured by the cJADAS-10, was inversely correlated with the first 25-OHD level (β=−0.20, 95% CI −0.37,–0.03, p=0.018), especially in 141 DMARD-naive patients (β=−0.26, 95% CI −0.44,–0.01, p=0.041). Up to the 3-year-follow-up, 17% (61/360) developed uveitis, and 30% (52/173) of OA patients an extended OA. While 20% (17/87) of those with 25-OHD deficiency at both measurements were affected by uveitis, this applied to only 9% (2/23) in those with sufficient levels. Multivariable regression analysis revealed that the 25-OHD level was significantly associated with the risk to develop uveitis (Hazard ratio 0.95, 95% CI 0.91,0.99, p=0.008). Twelve out of 29 (41%) patients with OA and two deficient vitamin D levels, but only 2 of 14 (14%) with two sufficient 25-OHD levels developed extended OA during the first 3 years (p=0.034). Conclusions Overall, vitamin D levels were higher in JIA patients than in matched controls. JIA patients with higher disease activity had lower 25-OHD levels. In particular, complicated courses with uveitis or the development of polyarthritis seem to be associated with lower 25-OHD levels. Further studies are needed to substantiate these results. Acknowledgements ICON is funded by the Federal Ministry of Research (FKZ:01ER0812) Disclosure of Interest C. Sengler: None declared, J. Zink: None declared, J. Klotsche: None declared, D. Foell: None declared, M. Niewerth: None declared, I. Liedmann: None declared, G. Horneff Grant/research support from: Abbvie, Pfizer, Roche, Chugai, MSD, J.-P. Haas: None declared, K. Moenkemoeller: None declared, A. Thon: None declared, K. Minden Grant/research support from: Pfizer, Abbvie, Roche

In der differenzialdiagnostischen Abklarung von Arthralgien und/oder Arthropathien bei Kindern und Jugendlichen sind auch einige z. T. sehr seltene monogenetische Erkrankungen in Betracht zu ziehen. Zunachst autoinflammatorische Erkrankungen wie das Cryopyrin assoziierte periodische Syndrom (CAPS), die pyogene Arthritis mit Pyoderma gangranosum und Akne (PAPA-Syndrom) und die Infantile Sarkoidose (Blau Syndrom); daruber hinaus genetisch bedingte Veranderungen in Regulations- und Strukturgenen des Bindegewebes wie die Progressive Pseudorheumatische Arthropathie des Kindesalters (PPAC), das Camptodaktylie-Arthropathie-Coxa vara-Perikarditis-(CACP) – Syndrom, die Multizentrische Carpotarsale Osteolyse (MCTO), die Spondyloenchondrodysplasie (SPENCD) und das Camurati-Engelmann Syndrom (CAEND). Die Vorliegende Ubersicht im Rahmen des Schwerpunktheftes gibt einen Uberblick uber diese seltenen Erkrankungen.

Die Versorgung rheumaerkrankter Kinder und Jugendlicher hat in den letzten Jahren erhebliche Fortschritte gemacht. Hierbei spielen mehrere Faktoren eine Rolle: (i) Verbesserung in der medikamentosen Therapie, (ii) Strukturelle Verbesserungen in der kinderrheumatologischen Versorgung, (iii) Multidisziplinare Therapiekonzepte und strukturierte Patientenschulung, (iv) Weiterentwicklung der funktionellen Therapien. Im Folgenden werden die wesentlichen Entwicklungen am Beispiel der Behandlung der Juvenilen Idiopathischen Arthritis in einer Ubersicht zusammengefasst.

Background Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated with the IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, disease course and adverse effects in a real-life clinical setting. Methods In 2009 a clinical and research consortium was established, including an online registry for autoinflammatory diseases (AID) (https://aid-register.de). Data for this retrospective TCZ study were documented by 13 centers. Results From 7/2009 to 4/2014, 200 patients with sJIA were recorded in the AID-registry. Out of these, 46 (19 m, 27 f, age 1–18 years) received therapy with TCZ. Long term treatment (median 23 months) has been documented in 24/46 patients who were evaluated according to Wallace criteria (active disease 6/24, inactive disease 5/24, remission 13/24 cases). Under observation co-medication were used in 40/46 cases. Adverse events were reported in 11/46 patients. The clinical response rate (no clinical manifestation, no increased inflammation parameters) within the first 12 weeks of treatment was calculated to be 35%. Conclusion Out of 200 sJIA children reported in the German AID-registry, 46 were treated with TCZ, showing a clinical response rate of 35% during the first 12 weeks, and inactive disease and/or remission under medication in 75% after one year. Adverse events were seen in 24% and severe adverse events in 4%. Trial registration The AID-Registry is funded by the BMBF (01GM08104, 01GM1112D, 01GM1512D).

OBJECTIVE: To determine whether systemic juvenile idiopathic arthritis (JIA) susceptibility loci that were identified by candidate gene studies demonstrate association with systemic JIA in the largest study population assembled to date. METHODS: Single-nucleotide polymorphisms (SNPs) from 11 previously reported systemic JIA risk loci were examined for association in 9 populations, including 770 patients with systemic JIA and 6,947 controls. The effect of systemic JIA-associated SNPs on gene expression was evaluated in silico in paired whole genome and RNA sequencing data from the lymphoblastoid cell lines (LCLs) of 373 European subjects from the 1000 Genomes Project. Responses of systemic JIA-associated SNPs to anakinra treatment were evaluated in 38 US patients for whom treatment response data were available. RESULTS: We found no association between the previously reported 26 SNPs and systemic JIA. Expanded analysis of the regions containing the 26 SNPs revealed only 1 significant association: the promoter region of IL1RN (P < 1 × 10(-4) ). Systemic JIA-associated SNPs correlated with IL1RN expression in LCLs, with an inverse correlation between systemic JIA risk and IL1RN expression. The presence of homozygous IL1RN high expression alleles correlated strongly with a lack of response to anakinra therapy (odds ratio 28.7 [95% confidence interval 3.2-255.8]). CONCLUSION: In our study, IL1RN was the only candidate locus associated with systemic JIA. The implicated SNPs are among the strongest known determinants of IL1RN and interleukin-1 receptor antagonist levels, linking low expression with increased systemic JIA risk. Homozygous high expression alleles predicted nonresponsiveness to anakinra therapy, making them ideal candidate biomarkers to guide systemic JIA treatment. This study is an important first step toward the personalized treatment of systemic JIA.

Background There is increasing experience with Etanercept (ETA) in juvenile idiopathic arthritis in the BIKER Registry. Objectives To report on practice changes ETA utilization and outcome over a period of 16 years. Methods 6 cohorts of pts were created according to inclusion period. Patients9 and disease characteristics,the utilization of DMARDs, steroids, NSAIDs were analysed. Efficacy was judged by PedACR30/50/70/90, JADAS and ACR-remission. Results Records from 2105 JIA pts treated with etanercept with at least a baseline and one follow up form were analysed. Most pts were females (67%). The median age of disease onset increased from 5.9 years in the early to 9.3 years in the later cohorts while age at start of treatment remained stable (about 13 years). Median disease duration markedly decreased from 5.3 to about 2 years. Most pts had RF neg. polyJIA followed by extended oligoarthritis. In the more recent cohorts the rate of enthesitis related arthritis increased and the rate f systemic JIA decreased (table). At registry start, 20% of newly enrolled pts belonged to the systemic JIA category compared to Conclusions In recent years, children have been treated earlier, received less concomitant treatment with NSAIDS, corticosteroids as well as DMARDs. More recent cohort of patients had less severe disease at baseline, but also showed a markedly better outcome already at one year of treatment reflected by higher rates of patients with no active joint, a CHAQ DI of 0, a JADAS-MDA, and ACR-Remission. These data suggest that early disease control and better pre-selection of patients who need biologics are important to improve outcome and safety in children with JIA. Disclosure of Interest G. Horneff Speakers bureau: Abbvie, Pfizer, Roche, Novartis, MSD, K. Minden Speakers bureau: Abbvie, Pfizer, Roche, Genzyme, Pharm-Allergan, I. Foeldvari: None declared, G. Ganser: None declared, J. Haas: None declared, J. Brunner: None declared, I. Becker: None declared

Background Since the approval of Adalimumab (ADA) for treatment of juvenile idiopathic arthritis (JIA), it has become a valuable option, which significantly improved the outcome of patients. Objectives To report efficacy (as observed) and safety of ADA in clinical practice. Methods Data from the German BiKeR register from 2011 to 2016 are reported. Baseline patient characteristics, treatment response and safety data were compared. Treatment response was analyzed using JIA-ACR criteria, JADAS score and improvement of functional status (Childhood Health Assessment Questionnaire disability index, CHAQ), JIA-ACR-scores, JADAS10-minimal disease activity (MDA), JADAS-remission and ACR-inactive disease criteria were analysed. Results 589 non-systemic JIA patients exposed to Adalimumab with at least one follow-up report were identified in the German BIKER registry, representing 1143.9 patient years (PY) of exposure to ADA and 1206.5 observation years including 90 days after discontinuation. At Baseline, 58.2% received combination with methotrexate (MTX). Patients on combination treatment had more frequently ANA, less frequently HLA-B27, had higher JADAS 10 and more often received systemic steroids (9.9% vs. 22.2%, p=0.0002). ADA dosage was 0.8mg/kg in both cohorts. At month 12 JIA-ACR 30/50/70/90 and JADAS-MDA/-remission/ACR-inactive disease was reached by 66/63/49/33/50/24/27% on monotherapy with ADA and 68/63/45/28%/50/28/27 on combination of ADA and MTX (not significant). Response rates at month 24 were 67/66/54/35/65/32/29% on ADA monotherapy and 67/61/46/31/57/35/30% on ADA+MTX combination cohort (not significant). The rate (/100PY) of all adverse events (AE)/serious AE/infections/serious infections/uveitis events was 52.3/2.2/16.4/4.6 upon monotherapy and 65.3/5.1/2.3/16.4/6.2 upon combination. Only rates for any AE were significantly higher upon combination (p=0.008) as well as rate of elevated transaminase (p=0.01). Rate of patients with an uveitis event was higher upon combination (9.6% vs. 5.3%, p=0.007) Conclusions Adalimumab demonstrated high response rates and an acceptable risk profile. Efficacy and safety of monotherapy was not inferior to combination therapy with MTX. Disclosure of Interest A. Klein: None declared, K. Minden Speakers bureau: Abbvie, Pfizer,Roche,Genzyme,Pharm-Allergan, I. Foeldvari: None declared, G. Ganser: None declared, J. Haas: None declared, R. Trauzeddel: None declared, A. Hospach: None declared, F. Weller-Heinemann: None declared, G. Horneff Grant/research support from: Abbvie, Chugai, Novartis, Pfizer,Roche

Definition Schmerzen, die ohne anhaltendes Trauma langer als 3 Monate ununterbrochen andauern, werden als chronische Schmerzen bezeichnet.

Hintergrund Die Glukokortikoidtherapie beim Kawasaki-Syndrom wird seit Jahren kontrovers diskutiert.

In der Rheumatologie zeigt der zunehmende Einsatz von Kombinationstherapien aus „disease modifying drugs“ (DMARDs) und Biologika auch bei schwer verlaufenden rheumatischen Grunderkrankungen im Kindes- und Jugendalter Erfolge. Diese Strategie steigert jedoch die medikamentenspezifischen infektiologischen Risiken. Zusatzlich bedingen viele Erkrankungen per se bereits ein erhohtes Infektionsrisiko. Einige Patienten weisen weitere immunologische oder organische Komorbiditaten auf, wie beispielsweise einen Komplementmangel oder eine pulmonale Gerusterkrankung, wodurch die Infektanfalligkeit zusatzlich steigt. Die hier vorgeschlagene Checkliste mit gezielten apparativen und immunologischen Vorsorgeuntersuchungen basiert auf einer „State-of-the-art-Auswertung“ der verfugbaren Literatur und eigenen Erfahrungen. Sie soll helfen, Risikofaktoren aufzudecken. Eine zusammenfassende Beurteilung von Grunderkrankung, Komorbiditaten und Wirkungsweise der Medikation ermoglicht somit 1) eine individuelle Risikostratifizierung der geplanten Immunsuppression und 2) eine Beurteilung der infektiologischen Gefahrdung des Patienten.

The goal of modern antirheumatic therapy is to achieve an optimized disease control. This is individually achieved by an intensified immunosuppression (IS) frequently combining different immunosuppressive agents. Intensified IS should be accompanied by a standardized protocol to monitor immunological changes in the patient. This should include checklists (see Part 1 Screening during intensified IS in children and adolescents). An individual risk stratification according to the planned IS allows a prediction of infectious disease risks for the patient and, thus, individual infection prophylaxis. In addition, standardized management of patients with fever while receiving intensified IS may prevent further complications.

Chronic pain syndromes in children and adolescents are defined as continuous or recurrent pain without an underlying causative diagnosis and lasting for more than 3 months. It is estimated that every fourth child in Germany suffers from chronic pain with every twentieth suffering from extreme recurrent pain. The incidence of chronic pain in children and adolescents is increasing with headache, abdominal pain and musculoskeletal pain being the most frequent. The quality of life declines not only due to the pain but to relieving postural and psychological factors, such as fear and sadness. School attendance, social activities and hobbies are mostly affected. This review summarizes the background of chronic pain syndromes and introduces a multimodal therapeutic approach.

Congenital anomalies of the kidneys and urinary tract (CAKUT) are among the most common anomalies in newborn infants, and may cause chronic renal disease in newborns. We investigated the sensitivity and specificity of different ultrasound-based screening strategies for CAKUT.Newborns (n = 4331) were analyzed for CAKUT in at least one ultrasound examination as a part of the Survey of Neonates in Pomerania (SNiP), a 7-year population-based study on neonates in Western Pomerania (Germany). Intrauterine ultrasound examinations were compared with early postnatal ultrasound findings (from days 3 - 7 of life) and pathological findings within the first 6 months of postnatal life.Cases of CAKUT were detected in 309 (3.7 %) kidneys in one ultrasound examination at the following points of time at least: (i) prenatally in 56 newborns (18.2 %), (ii) 3 - 7 days postnatally in 201 newborns (65.2 %) and (iii) in 52 newborns (17 %) during the 6-month follow-up. The prevalence was significantly higher in male infants, and hydronephrosis was found to be the most frequent obstructive nephropathy (83.3 %). Significant co-morbidity was observed with CNS malformations. The diagnostic sensitivity was significantly higher in postnatal ultrasound screening (79.6 vs. 18.2 % prenatally), while the specificity was above 99 % at all time points.This study demonstrates a high prevalence of CAKUT and demonstrates the importance of combined prenatal and postnatal ultrasound examinations for early CAKUT diagnosis.ZIEL:: Angeborene Fehlbildungen der Nieren und ableitenden Harnwege (CAKUT) gehören zu den häufigsten neonatalen Anomalien und können zur Entstehung chronischer Nierenerkrankungen und Niereninsuffizienz führen. In dieser Studie haben wir Sensitivität und Spezifität der verschiedenen ultraschallbasierten Screening-Ansätze untersucht.Im Rahmen des über 6 Jahre durchgeführten Survey of Neonates in Pomerania (SNiP) erhielten Neugeborene (n = 4331) mindestens eine Ultraschalluntersuchung der Nieren und ableitenden Harnwege im Rahmen der zweiten Vorsorgeuntersuchung (U2) zwischen dem 4.–7. Lebenstag. Intrauterine Ultraschallergebnisse wurden mit den postnatalen Ergebnissen und eventuellen weiteren Auffälligkeiten in den ersten 6 Lebensmonaten verglichen.Bei 309 (3,7 %) Nieren wurde in einer der folgenden Ultraschalluntersuchungen eine CAKUT diagnostiziert, (i) pränatal bei 56 Neugeborenen (18,2 %), (ii) 4 – 7 Tage postnatal bei 201 (65,2 %) und (iii) während der ersten 6 Lebensmonate bei weiteren 52 (17 %) Neugeborenen. Es zeigte sich eine signifikant höhere Prävalenz bei männlichen Neugeborenen. Eine angeborene Hydronephrose war die am häufigsten gefundene obstruktive Nephropathie (83,3 %). Eine signifikante Komorbidität zeigte sich mit Auffälligkeiten des ZNS. Die diagnostische Sensitivität des postnatalen Screenings war signifikant höher (79,6 vs. 18,2 % pränatal), die Spezifität war zu allen Screeningzeitpunkten über 99 %.Diese Studie zeigt eine hohe CAKUT-Prävalenz und demonstriert die Wichtigkeit eines Ultraschallscreenings zur Früherkennung einer angeborenen Fehlbildung der ableitenden Harnwege.

Juvenile idiopathic arthritis (JIA) summarizes a group of inflammatory diseases of childhood. The etiology remains still unclear. In JIA, T cells have been demonstrated to play key roles in the pathogenesis. T-cell proliferation in JIA may be different in the peripheral blood (PB) and the synovial fluid (SF). The aim of this study is to demonstrate the turnover of T cells in the PB and SF of patients with persistent oligoarticular JIA (oJIA) compared to controls. Matched pairs of samples were investigated derived from PB and SF of nine patients with persistent oJIA. The cells from PB and SF were determined by flow cytometry. The majority of the PBMC and IAMC were in phase G0/G1, with fewer than 1% in S phase. In the SF, the percentage of cells in the S phase are higher than in the PB. The percentage of cells in the S phase in SF are equal to the result in the control group. In conclusion, the turnover of synovial T cells in persistent oJIA is higher than in the PB.

Inhibitoren von Tumornekrosefaktor α (TNFα) und andere Biologika haben die Therapieoptionen bei juveniler idiopathischer Arthritis erheblich erweitert. Die Wirksamkeit von Etanercept und Adalimumab wurde in randomisierten kontrollierten klinischen Studien nachgewiesen. Die Langzeitsicherheit bleibt Gegenstand laufender Untersuchungen. Die U.S. Food and Drug Administration berichtete im August 2009 uber insgesamt 48 bosartige Tumoren bei mit Infliximab, Etanercept oder Adalimumab behandelten Kindern und Jugendlichen, was Fragen bezuglich eines erhohten Malignomrisikos aufwirft. Lymphome machten mit 50% den grosten Anteil der 48 Malignome aus. Die Indikation fur die Anwendung von TNF-Inhibitoren ist unter Berucksichtigung von Risikofaktoren sorgfaltig zu prufen. Dies gilt insbesondere fur nicht zugelassene Substanzen, nicht zugelassene Indikationen und fur Kombinationsbehandlungen mit TNF-Inhibitoren und Immunsuppressiva. Andererseits soll aufgrund der aktuellen Datenlage bei keinem Patienten, der die entsprechenden Voraussetzungen erfullt, eine notwendige Therapie beendet oder nicht begonnen werden. Eine adaquate Aufklarung, Uberwachung und Langzeitdokumentation der Behandlung im Register der Gesellschaft fur Kinder- und Jugendrheumatologie werden dringlich empfohlen.

Neonatal health is of major concern to parents, midwives, physicians and society as a whole, yet a prospective population-based birth cohort to collect comprehensive data on multiple issues including medical, social, environmental and genetic aspects remains to be established in Germany. The survey of newborns in Pomerania (SNiP) described in this paper attempts to take up this goal. The objectives of SNiP are to establish (a) a population-based birth cohort providing detailed information about neonatal health, morbidity and mortality, (b) a biobank with newborn DNA and serum from cord blood, placenta tissue samples and DNA obtained from oral mucosal swabs of the mothers, (c) a prospective study design by re-examination of the SNiP population prior to attendance at primary school. From March 2003 until November 2008 all childbearing mothers in a well-defined region in North-Eastern Germany were asked to participate with their newborns. Detailed data on health status of the newborn, pregnancy, medical and family history, socio-economic status and maternal life style were obtained via face-to-face interview, standardised questionnaires and medical records. Placental tissue samples, cord blood plasma and DNA were continuously collected; sampling of maternal DNA from mouth swabs started in 2007. As a result, during the study period n = 6747 births and n = 6828 babies were enrolled. A population coverage of 95% was achieved. The active participation rate was 75%. A non-responder analysis revealed no meaningful selection bias. Thus, SNiP is a population-based, representative study in Germany that is able to describe the health and living conditions of newborns and their families comprehensively. It can contribute to existing knowledge and to similar cohort studies since data are accessible by researchers.

ZusammenfassungDie juvenile Psoriasis-Arthritis (jPsA) wird als eine Kategorie der juvenilen idiopathischen Arthritis (JIA) definiert und weist unterschiedliche klinische Manifestationsformen auf. Die größte Gruppe bilden Patienten mit einer asymmetrischen Arthritis im Schulalter, gefolgt von Kindern mit Oligoarthritis und Beginn im Kleinkindalter. Bei zehn bis 20 Prozent besteht eine symmetrische Polyarthritis. In der Klassifikation nach ILAR von 2002 gelten Ausschlusskriterien, die insbesondere Jungen mit einer Spondylarthropathie betreffen. Dadurch ergeben sich Schwierigkeiten im Vergleich mit früheren Studien sowie bei Lanzeitstudien. Die Spondylitis psoriatica stellt eine typische Manifestation der adulten Psoriasis-Arthritis dar. Die Erkrankung dieser Patienten muss im Kindesalter jedoch oft als undifferenzierte Arthritis klassifiziert werden. Die Therapie der jPsA erfolgt multidisziplinär entsprechend den Richtlinien der JIA. Die medikamentöse Behandlung schließt neben der dermatologisch topischen Versorgung die Gabe von NSAR, intraartikuläre Steroidinjektionen und je nach Verlauf auch Basismedikamente und/oder Biologika mit ein. Wichtige Bestandteile der Therapie sind Physio- und Ergotherapie sowie Hilfsmittelversorgung. Im Hinblick auf den chronischen Verlauf ist auch eine psychosoziale Betreuung von Kind und Familie erforderlich. Primär muss von einem lebenslänglichen Verlauf ausgegangen werden, weshalb beim Übergang ins Erwachsenenalter auch eine betreute Transition der Patienten in die Erwachsenenrheumatologie erfolgen sollte. Die Prognose hängt entscheidend von einer frühzeitigen konsequenten Therapie ab. Anhaltenden Remissionen stehen progredient destruktive Verläufe gegenüber. Neben einer Übersicht zum Thema werden Daten aus den Langzeitverläufen von 79 Patienten mit einer jPsA vorgestellt und diskutiert. Die ILAR-Klassifikation der jPsA bietet eine gute Klassifikationsgrundlage, definiert jedoch einige Ausschlusskriterien, die angesichts unserer Analysen zuviele Patienten aus dieser Gruppe ausgrenzen.

BACKGROUND: The official birth statistics are regarded as a reliable data source on births and birth rate in the German population. However, they show methodological limitations with respect to the identification of first-time mothers and the number of children per mother. The mothers’ social and economical background is not assessed. The goal of the present analysis was (a) to describe demographic and socio-economic variables of all births in a defined region over a fixed time-frame and (b) to make a comparison on the basis of parity and gravidity. METHOD: From 2004-2007 4 982 children were born in the region and data from n=4 788 children were assessed (96%); n=3 505 (73%) of these mothers consented to a more detailed assessment. RESULTS: The fertility rate in the SniP region is low. There are fewer children per 1 000 women and born per women in general. The average age of primiparae was 25 and 26 years. As can be expected there is a significant difference between primiparae and multiparae with respect to age. There is also a difference in occupational status. 17% of the primiparae have been multigravidae. CONCLUSIONS AND DISCUSSION: For the first time in Germany, the SNiP collected comprehensive population-based data on the age and socio-demographic variables of children and their mothers in a defined geographical region. A significant discrepancy for average age of primiparae between the study results and the official statistics is discussed in the light of methodological and regional issues. Our results require the continuation of comprehensive population-based data assessment. Furthermore, the SniP region could serve as a model region for future research. In international comparisons Germany?s reproductive behaviour has proved to be unfavourable, which is accentuated in the region under examination.