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      IgG4-related disease: an update on pathophysiology and implications for clinical care

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      Nature Reviews Rheumatology
      Springer Science and Business Media LLC

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          Abstract

          <p class="first" id="d1535732e79">IgG4-related disease (IgG4-RD) has only existed as a unique disease entity since 2003, yet remarkable progress has already been achieved in describing the essential features of the disease. A framework for systematic clinical studies has been created by the development of a quantitative disease activity tool (the IgG4-RD Responder Index) and the validation of classification criteria, both of which were the products of international, multi-centre investigations. In addition, substantial strides have been made in understanding the pathophysiology of IgG4-RD. In particular, the central role of B cells in the disease has been demonstrated by both the robust clinical responsiveness of IgG4-RD to B cell depletion and by the identification of multiple self-antigens that promote B cell expansion. CD4+ T cells have also been investigated in detail; CD4+ cytotoxic T lymphocytes (suspected of promoting disease) and a specific T follicular helper cell subset that contributes to IgG4 isotype switching have both been defined by multiple groups. The mechanisms by which these immune cells converge on target tissues, interact with fibroblasts and promote tissue remodelling are beginning to be understood and will be an important research focus in the coming years. </p>

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          Author and article information

          Journal
          Nature Reviews Rheumatology
          Nat Rev Rheumatol
          Springer Science and Business Media LLC
          1759-4790
          1759-4804
          September 16 2020
          Article
          10.1038/s41584-020-0500-7
          32939060
          a5c63bd9-df1c-4631-86d1-6dc3e03880a7
          © 2020

          http://www.springer.com/tdm

          http://www.springer.com/tdm

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