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IgG4-related disease (IgG4-RD) is a relapsing–remitting immune-mediated fibroinflammatory disease characterised by: a tendency to mass forming, tissue-destructive lesions in multiple sites; a prompt response usually to steroids; a characteristic histopathological appearance; and elevated serum IgG4 concentrations in approximately 60–70% of people during an acute phase.〔〔 The disease, which was formerly also known as IgG4-related systemic disease, is so named because plasma cells producing the antibody subtype IgG4 are present in large amounts on tissue samples from involved organs, and because IgG4 antibody levels in the bloodstream are often elevated. Inflammation results in fibrosis, the deposition of connective tissue, in affected anatomical sites which can lead to organ dysfunction, or even organ failure, if not treated.〔 Early detection is important to avoid organ damage and potentially serious complications.〔 ==Nomenclature== Prior to 2011, IgG4-RD used to get mentioned in the medical literature under various different names.〔〔 At the (2011 International Symposium on IgG4-Related Diseases ), the consensus name of IgG4-related disease was endorsed for the condition.〔 This name had already been agreed upon as a consensus name among Japanese investigators,〔〔 notably choosing not to use the term 'systemic' as that might lead to malignant tumours in other organs getting incorrectly diagnosed as being just another manifestation of the IgG4-related condition.〔 However, some experts at the international symposium did express reservations about naming the disease after IgG4, as its role in pathogenesis is questionable and the use of serum IgG4 concentrations as a biomarker is unreliable.〔 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「IgG4-related disease」の詳細全文を読む スポンサード リンク
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