
IgG4-related disease (IgG4-RD) is a complex, multi-organ fibro-inflammatory condition with overlapping features seen in other immune disorders. Diagnosis is often difficult due to lack of validated criteria, requiring a multi-dimensional approach. This review highlights recent advances in pathogenesis, clinical mimickers like histiocytoses and vasculitides, and evolving treatments. New therapies targeting B cells and cytokines offer promise beyond traditional glucocorticoids in ongoing clinical trials.
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