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One-third of genetically confirmed monogenic lupus patients remained unclassified, particularly those with interferon-related and nucleic acid metabolism defects, highlighting pathway-dependent limitations of current classification criteria.
OBJECTIVES To compare the clinical, immunological, and diagnostic characteristics of childhood-onset (coSS) versus adult-onset Sjögren syndrome (aoSS), assess disease activity and damage accrual, and identify independent predictors of organ damage. METHODS This national, multicenter retrospective cohort included 197...
The findings highlight a regional male predominance and a paradigm shift toward MRI-based diagnosis, reducing the need for invasive biopsies, and the high utilization of biologic therapies underscores the refractory nature of the disease in tertiary care settings.
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