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Molecular Pathways in the Pathogenesis of Systemic Juvenile Idiopathic Arthritis
Ist Teil von
Rheumatic diseases clinics of North America, 2023-11, Vol.49 (4), p.895-911
Ort / Verlag
United States: Elsevier Inc
Erscheinungsjahr
2023
Link zum Volltext
Quelle
MEDLINE
Beschreibungen/Notizen
Systemic juvenile idiopathic arthritis (sJIA) is a rare childhood chronic inflammatory disorder with risk for life-threatening complications including macrophage activation syndrome and lung disease. At onset, sJIA pathogenesis resembles that of the autoinflammatory periodic fever syndromes with marked innate immune activation, expansion of neutrophils and monocytes, and high levels of interleukin-18. Here, we review the current conceptual understanding of sJIA pathogenesis with a focus on both innate and adaptive immune pathways. Finally, we consider how recent progress toward understanding the immunologic basis of sJIA may support new therapies for refractory disease courses.