IgG4-related Disease - A Systemic Disease that Deserves Attention Regardless of One's Subspecialty

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Author(s)

    • Hamano Hideaki
    • Division of Medical Informatics, Shinshu University Hospital, Japan|Department of Internal Medicine, Gastroenterology, Shinshu University School of Medicine, Japan
    • Tanaka Eiji
    • Department of Internal Medicine, Gastroenterology, Shinshu University School of Medicine, Japan
    • Kawa Shigeyuki
    • Department of Internal Medicine, Matsumoto Dental University, Japan

Abstract

<p>IgG4-related disease (IgG4-RD) is an inflammatory condition characterized by a high serum IgG4 concentration and the abundant infiltration of lymphocytes and IgG4-positive plasma cells in the tissue, as well as spatial (diverse clinical manifestations) and temporal (the possibility of recurrence) multiplicities. Since the initial documentation of IgG4-related disease in patients with autoimmune pancreatitis in 2001, a growing body of evidence has been accumulating to suggest that various-virtually all-organs can be affected by IgG4-RD. In general, steroid therapy is effective and is considered to be the first-line treatment for IgG4-RD. The precise mechanism underlying this systemic disorder has remained unknown. Considering that IgG4-RD was specified as being an intractable disease in 2015, further studies are needed to clarify whether IgG4-RD is indeed a distinct disease entity or a complex of disorders of different etiologies and clinical conditions. </p>

Journal

  • Internal Medicine

    Internal Medicine 57(9), 1201-1207, 2018

    The Japanese Society of Internal Medicine

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