Article Text

Download PDFPDF
Why is one arm stronger than two arms? IgG4 antibodies in IgG4-related autoimmune pancreatitis
  1. Julia Mayerle1,
  2. Markus M Lerch1,
  3. Andreas Greinacher2
  1. 1Department of Medicine A, University Medicine, Ernst-Moritz-Arndt-University Greifswald, Greifswald, Germany
  2. 2Institute of Immunology and Transfusion Medicine, University Medicine, Ernst-Moritz-Arndt-University Greifswald, Greifswald, Germany
  1. Correspondence to Professor Julia Mayerle, Department of Medicine A, University Medicine, Ernst-Moritz-Arndt-University, Greifswald, Ferdinand-Sauerbruchstrasse, Greifswald 17475, Germany; mayerle{at}

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

In this issue, Shiokawa et al1 recapitulated the approach, previously established in pemphigus vulgaris,2 of passive transfer of IgG1 and IgG4 purified from patients with autoimmune pancreatitis (AIP) type 1 into neonatal Balb/c mice. These patients suffered from IgG4-related disease (IgG4-RD).

IgG4-RD is a novel clinical entity that includes a growing number of medical conditions that have the following features in common: diffuse organ swelling or focal mass formation, sclerosing storiforme (whirl-like) fibrosis with a lymphoplasmacytic infiltrate rich in IgG4-bearing plasma cells and elevated levels of serum IgG4. The disease usually responds well to steroid treatment and is predominantly diagnosed in elderly men.3 ,4 IgG4-RD is poorly understood but is currently classified as an autoimmune disease because of its association with autoantibody formation. However, so far there has been no evidence that the autoantibodies reported in IgG4-RD contribute directly to the pathogenesis.5 In many autoimmune disorders, which are unequivocally T-cell-driven, autoantibodies such as antinuclear antibodies and rheumatoid factor are detected, but such antibodies are not necessarily responsible for the tissue damage resulting in autoimmune disease, which is rather primarily induced by T cells. While T cells are likely also important for IgG4-RD (although it is still debated whether Th1 or Th2 cytokines ultimately drive the disease process6 ,7), the present study provides solid evidence that in IgG4-RD-associated AIP IgG4 is more than just a biomarker, or an epiphenomenon, but a driver and also a modulator of the disease.

Total IgG from patients with IgG4-RD resulted in injury to the pancreas and salivary glands of the mice. …

View Full Text


  • Contributors JM, MML and AG have jointly drafted and reviewed the commentary submitted here.

  • Funding This work was supported by the Deutsche Krebshilfe/Dr. Mildred-Scheel-Stiftung (109102), the Deutsche Forschungsgemeinschaft (DFG MA 4115/1-2/3), the Federal Ministry of Education and Research (BMBF GANI-MED 03IS2061A and BMBF 0314107, 01ZZ9603, 01ZZ0103, 01ZZ0403, 03ZIK012) and the European Union (EU-FP-7: EPC-TM).

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

Linked Articles

  • Pancreas
    Masahiro Shiokawa Yuzo Kodama Katsutoshi Kuriyama Kenichi Yoshimura Teruko Tomono Toshihiro Morita Nobuyuki Kakiuchi Tomoaki Matsumori Atsushi Mima Yoshihiro Nishikawa Tatsuki Ueda Motoyuki Tsuda Yuki Yamauchi Ryuki Minami Yojiro Sakuma Yuji Ota Takahisa Maruno Akira Kurita Yugo Sawai Yoshihisa Tsuji Norimitsu Uza Kazuyoshi Matsumura Tomohiro Watanabe Kenji Notohara Tatsuaki Tsuruyama Hiroshi Seno Tsutomu Chiba