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Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Localized scleroderma is an autoimmune disease belonging to the group of collagenoses, which can manifest cutaneously and extracutaneously. The extracutaneous manifestations may have significant morbidity but are not considered in previous scoring systems. For this reason, another scoring system, the total morbidity score (TMS) was developed, which also takes into account the extracutaneous symptoms., Method: In the retrospective monocentric study at the Hamburg Center for Pediatric and Adolescent Rheumatology, the TMS was applied to patients from 2004-2019 suffering from localized scleroderma who had at least one control presentation. In addition, data were analyzed according to the previously established localized scleroderma cutaneous assessment tool (LoSCAT) scoring systems to ensure better comparability to the TMS. Furthermore, the score values were considered and compared during the course of treatment with methotrexate (MTX)., Results: Due to a lack of control presentations, data from 51 of the 95 patients with a confirmed diagnosis could be included in the retrospective evaluation. The treatment of these patients was considered over a period of 2 years, from the initial presentation over at least 3 further control presentations. The TMS total score remained largely constant. There was a weak correlation between the TMS total score and the localized scleroderma skin damage index (mLoSDI), which indicates the degree of damage. In addition, insignificant changes in the TMS total score were shown over time with MTX treatment (T1/T4: -0.007)., Discussion: The evaluation showed that the TMS total score is mainly fed by the extracutaneous manifestations, demonstrating the inaccuracy of previous scores. Another advantage of the TMS is that different scores are assigned depending on whether the feature is new, persistent, improving, or even worsening. The TMS is more time consuming to collect but enables a more accurate assessment of disease activity., (© 2022. The Author(s).)

Juvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of myositis-specific autoantibodies (MSA). In contrast to dermatomyositis in adults, in children and adolescents there are no associations with malignant diseases. The variable expression, the rarity of the disease and the risk of long-term damage and complications necessitate pediatric rheumatological experience in the diagnostics and treatment. Recently, new approaches in drug treatment have substantially improved the outcome and prognosis but a multidisciplinary treatment (including physicians, physiotherapists, psychologists, social workers) is mandatory, especially in the first phases of the disease. Particularly important is a professionally correct treatment of the functional sequelae, which are a particular focus of this article., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

We created a registry to evaluate long term outcome, efficacy and adverse events for children treated wit TNF-α inhibitors in Switzerland. 106 patients (68 female/38 male) were included. 61 patients were treated with Etanercept (Enbrel®) and 45 with Infliximab (Remicade®). Concomitant treatment at baseline included corticosteroids in 26% and Methotrexate in 75% of the patients. Subjective disease activity three months after initiation of TNF-α was better in 81%, worse in 4% and stable in 15% of the patients. In total 24 adverse events in 21 patients were reported. Treatment with TNF-α inhibitors seems to be safe and effective for children and adolescents with rheumatologic diseases. [ABSTRACT FROM AUTHOR]

Due to the underlying disease and immunosuppressive treatment, pediatric patients with rheumatic diseases are at increased risk for (long distance) travel-related health problems. A pretravel comprehensive consultation is therefore strongly recommended. Whether a child with rheumatic disease is sufficiently fit for travel essentially depends on the disease activity, the age of the child and the intended travel destination. Depending on the level of immunosuppression, the risks for this patient group include (travel-related) infections and the possibility of disease activity flares. Of particular importance is adequate exposure prevention: standard vaccinations should be updated and indications for travel vaccinations evaluated in advance of travelling. In this context, potential contraindications, especially for live vaccines, in the case of specific immunosuppressive treatment should be considered. In the event of travel to malaria endemic areas, the necessity for chemoprophylaxis or stand-by medication must be evaluated but caution is needed regarding potential drug interactions. Detailed education about careful hand, food and contact hygiene is critical. Because photosensitivity may be increased in some rheumatic diseases and/or medications, UV protection is crucial. Barriers (clothes and mosquito nets) and age-appropriate chemical insect repellents should be used to prevent insect-borne diseases. Before start of travel, possibilities for on-site medical help in the event of disease deterioration and/or infection should be evaluated. This should be included in the assessment of the patient's ability to travel. Travelers with rheumatic disease should carry a first aid kit that includes both a sufficient supply of regular antirheumatic medication and supplemental medication in case of a disease flare. Storage conditions must be taken ínto account for some medications. Ultimately, the success of a journey depends on the planning from the perspective of the child with its specific needs., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)