Immunoglobulin (Ig) G4-related disease is an immune-mediated fibro-inflammatory condition that can involve virtually every organ system. Our knowledge and understanding of this condition has expanded rapidly over the last decade, with insights into adaptive and innate immunological mechanisms, identification of novel risk factors, definition of disease subsets based on organ distribution, discovery of circulating biomarkers that correlate with disease activity and relapse, and novel treatment approaches.
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