Journal of Gastroenterology

, Volume 38, Issue 10, pp 982–984

A new clinicopathological entity of IgG4-related autoimmune disease

  • Terumi Kamisawa
  • Nobuaki Funata
  • Yukiko Hayashi
  • Yoshinobu Eishi
  • Morio Koike
  • Kouji Tsuruta
  • Atsutake Okamoto
  • Naoto Egawa
  • Hitoshi Nakajima
Rapid communication

Background

Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases.

Methods

To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies.

Results

In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancreatic tissue (6/6), bile duct (8/8), gallbladder (8/8), portal area of the liver (3/3), gastric mucosa (5/7), colonic mucosa (2/2), salivary glands (1/2), lymph nodes (6/6), and bone marrow (2/2), as well as in the pancreas (8/8). There were few IgG4-positive plasma cells at the same sites in controls.

Conclusions

These results suggest that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement. We propose a new clinicopathological entity, of a systemic IgG4-related autoimmune disease in which AIP and its associated diseases might be involved.

Key words

autoimmune pancreatitis IgG4 autoimmune disease 

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Copyright information

© Springer-Verlag Tokyo 2003

Authors and Affiliations

  • Terumi Kamisawa
    • 1
  • Nobuaki Funata
    • 2
  • Yukiko Hayashi
    • 2
  • Yoshinobu Eishi
    • 3
  • Morio Koike
    • 3
  • Kouji Tsuruta
    • 4
  • Atsutake Okamoto
    • 4
  • Naoto Egawa
    • 1
  • Hitoshi Nakajima
    • 1
  1. 1.Department of Internal MedicineTokyo Metropolitan Komagome HospitalTokyoJapan
  2. 2.Department of PathologyTokyo Metropolitan Komagome HospitalTokyoJapan
  3. 3.Department of PathologyTokyo Medical and Dental University School of MedicineTokyoJapan
  4. 4.Department of SurgeryTokyo Metropolitan Komagome HospitalTokyoJapan

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