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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.

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.

Impfungen stellen bei Kindern und Jugendlichen mit rheumatischen Erkrankungen ein besonderes Problem dar. Wirkungen und Nebenwirkungen von Impfungen sind fur dieses Patientenkollektiv leider nur unzureichend untersucht, und spezifische Impfempfehlungen fehlen. Die bei diesen Patienten haufig erforderliche immunsuppressive Therapie schafft zusatzliche Unsicherheit. Hinzu kommen Bedenken bezuglich impfassoziierter Reaktivierungen der Grunderkrankung. Die bestehenden Unsicherheiten im Umgang mit Impfungen fuhren zu einer erheblichen Praxisvariation unter den Kinderarzten und Impflucken bei den betroffenen Kindern und Jugendlichen. So ist jeder dritte Patient mit juveniler idiopathischer Arthritis unzureichend geimpft, was sogar Standardimpfungen mit Totimpfstoffen wie Tetanus/Diphtherie einschliest. Nach aktuellem Stand des Wissens ist der Nutzen vieler Impfungen gerade bei Patienten mit Autoimmunerkrankungen deutlich hoher als deren Risiko zu veranschlagen. Gerade Patienten mit immunsuppressiver Therapie benotigen einen besonderen Schutz vor Infektionen. Kinder und Jugendliche mit rheumatischen Erkrankungen sollten deshalb – soweit moglich – nach den STIKO-Empfehlungen geimpft werden. Dabei muss der Zeitpunkt der anstehenden Impfung sorgfaltig in Abhangigkeit von der Krankheitsaktivitat und Therapie gewahlt werden.

The increasing use of combination therapies, including disease-modifying antirheumatic drugs (DMARD) and biologicals has improved the outcome for children and adolescents in several rheumatic diseases. However, this strategy has increased the risk of drug-specific side-effects, such as an increased risk of infections. Furthermore, the underlying rheumatic disease itself often includes an increased risk of infections and some patients additionally present with immunological or organic comorbidities (e.g. complement deficiency and interstitial pulmonary disease) further increasing the susceptibility to infections. The presented review is based on an analysis of the currently available literature proposing a checklist of diagnostic procedures and immunological laboratory tests specific for the detection of patients prone to infections. The combined stratification of the underlying disease, comorbidities and the immunological mechanisms of the medication enables (1) an individual risk stratification of planned immunosuppressive therapy and (2) a prediction of the risks of infection for the patient.

Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

Several genetic factors have recently been observed as having an influence on susceptibility, course and prognosis of juvenile idiopathic arthritis (JIA): 1. Affected sib pairs were observed to have a low concordance in terms of disease incidence, but significant concordance in terms of subtype and course of disease. 2. Each subtype of JIA was observed to have a distinct genetic background. 3. Some JIA patients do not carry any of the defined risk genes. 4. Most subtypes of JIA have a distinct different genetic background to rheumatoid arthritis in adults. 5. Multiple factors have been observed to be involved in pathogenesis implying genetic and environmental factors. 6. Systemic JIA differs from all other subtypes in terms of genetic background and treatment options. It is currently assumed to be an autoinflammatory disease. 7. Genetic factors not only affect the course of the disease, but also response and complication rate. Increasing knowledge on the factors involved in the pathogenesis of JIA as well as analysis of large patient cohorts in consortiums cooperating on an international level have helped define many important polymorphisms; these are currently the subject of further investigation.

TNF inhibitors and other biologicals have greatly expanded the therapeutic options for juvenile idiopathic arthritis (JIA). While the efficacy of etanercept and adalimumab has been proven in randomized controlled clinical trials, their long-term safety remains the subject of ongoing investigations. Reports of leukaemia and tumours in children and adolescents treated with etanercept, infliximab and adalimumab have raised questions about an increased risk for malignancies, with lymphoma accounting for the largest group at 50% of all 48 malignancies reported by the FDA.Consequently, TNF inhibitors should be indicated under careful consideration of individual risk factors, such as increased family occurrence of malignancies, or pre-treatment with carcinogenic substances such as cyclophosphamide. This is particularly true for non-approved substances, and non-approved indications, and for combination therapy of TNF inhibitors with immunosuppressive drugs. On the other hand, however, treatment should not be stopped or started in any patient in whom treatment is necessary due to the current knowledge. Adequate patient information, surveillance and documentation of treatment in the registry of the GKJR is strongly recommended.

Vaccinations represent a special problem in children and adolescents with inflammatory rheumatic diseases. There are very limited data on the safety and efficacy of vaccines in these patients, and guidelines for immunization are missing. The immunosuppressive therapy often necessary for these patients gives rise to additional uncertainty. In addition, many colleagues consider vaccination to increase the risk of relapse of the rheumatic illness. As a consequence, there are substantial variations in practicing vaccination in these patients, resulting in insufficient vaccination coverage rates. For example, every third patient with juvenile idiopathic arthritis is incompletely vaccinated; this even includes toxoid vaccines for tetanus and diphtheria. The benefit of vaccinations, which far outweighs their potential risks, is well recognized even in patients with autoimmune diseases. These patients in particular require a special protection from infections due to their immunosuppressive therapies. Therefore, children and adolescents with rheumatic diseases should be immunized according to the Standing Immunization Commission of the Robert Koch Institute recommendations whenever possible. However, the time of vaccination must be carefully selected, taking disease activity and treatment into account.