An Overview

  • Shigeyuki Kawa
  • Mitsuhiro Kawano


Conferences focusing on IgG4-related disease started in 2007 in Japan comprising a small group of Japanese researchers, as a meeting on type 1 autoimmune pancreatitis (AIP) and the various types of extrapancreatic organ involvement. It raised the field to a higher status, and the attendees resolved to conduct annual meetings for the study of “IgG4-related disease (IgG4-RD).” Subsequently, intensive studies on IgG4-RD have been carried out worldwide. In this chapter, we focused on the establishment of the concept of IgG4-RD, with special reference to IgG4 as a subclass of IgG, conditions causing abnormal elevations of IgG4 or associated with IgG4, clinical picture of IgG4-RD, and mechanisms underlying its development.


Thrombotic Thrombocytopenic Purpura Bullous Pemphigoid Epidermolysis Bullosa Autoimmune Pancreatitis Epidermolysis Bullosa Acquisita 
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Copyright information

© Springer Japan 2014

Authors and Affiliations

  1. 1.Center for Health, Safety, and Environmental ManagementShinshu UniversityMatsumoto CityJapan
  2. 2.Division of RheumatologyKanazawa University HospitalKanazawaJapan

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