Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and the Berlin Institute of Health, 10117 Berlin, Germany; Deutsches Rheuma-Forschungszentrum (DRFZ Berlin), ein Leibniz Institute, 10117 Berlin, Germany.
Department of Women's and Children's Health, Institute of Live Course and Medical Sciences, University of Liverpool, Liverpool, UK; Department of Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
Clin Immunol. 2022 Jan;234:108907. doi: 10.1016/j.clim.2021.108907. Epub 2021 Dec 8.
Systemic lupus erythematosus (SLE) is a systemic autoimmune/inflammatory disease that can affect any organ system and cause significant damage and organ failure. Disease-onset during childhood (juvenile-onset SLE) is associated with less typical autoantibody patterns, diffuse organ involvement, more damage already at diagnoses, and a higher need of immunomodulating treatment, including corticosteroids, when compared to adult-onset SLE. Differences in the molecular pathophysiology within SLE, and over-representation of patients with "genetic SLE" contribute to differences in clinical presentation and treatment responses between children and adults. This manuscript summarizes currently available literature focusing on parallels and differences between clinical pictures, known pathomechanisms, and available treatment options in juvenile- versus adult-onset SLE.
系统性红斑狼疮（SLE）是一种全身性自身免疫/炎症性疾病，可影响任何器官系统并导致严重的损伤和器官衰竭。儿童期发病（幼年特发性红斑狼疮）与不太典型的自身抗体模式、弥漫性器官受累、诊断时已存在更多损伤以及更高的免疫调节治疗需求（包括皮质类固醇）相关，与成人发病的SLE 相比。SLE 内的分子病理生理学的差异以及“遗传 SLE”患者的过度表现导致儿童和成人之间在临床表现和治疗反应方面存在差异。本文总结了目前可获得的文献，重点关注幼年特发性和成人发病的 SLE 之间在临床特征、已知发病机制和可用治疗方案方面的相似和不同之处。
