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Serum IgG4-negative autoimmune pancreatitis

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

Background

Autoimmune pancreatitis (AIP) is considered to be a pancreatic lesion of IgG4-related systemic disease, but about 20% of AIP patients do not have elevated serum IgG4 levels. This study aimed to clarify the pathophysiology of AIP patients without elevated serum IgG4 levels.

Methods

Fifty-eight AIP patients were divided into 2 groups: those with elevated serum IgG4 levels (>135 mg/dl; SIgG4-positive AIP) and those without (SIgG4-negative AIP). The 2 groups’ clinical, serological, radiological, and histological findings, as well as salivary and lacrimal gland function, were compared.

Results

Serum IgG4 levels were elevated in 45 AIP patients and normal in 13 patients. In SIgG4-negative AIP patients, the female ratio tended to be high; obstructive jaundice was less likely; abdominal pain and acute pancreatitis were more likely; segmental swelling of the pancreatic body and/or tail was more common; sclerosing extrapancreatic lesions, salivary and lacrimal gland dysfunction, and abundant infiltration of IgG4-positive plasma cells in the gastric mucosa were less likely; and conservative follow-up was sometimes implemented. Histological examination of the pancreas of SIgG4-negative AIP showed lymphoplasmacytic sclerosing pancreatitis (LPSP) rather confined to the pancreas (n = 4), inadequate material (n = 2), and pancreatic fibrosis showing infiltration of lymphocytes without infiltration of IgG4-positive cells or neutrophils (n = 2).

Conclusions

Clinicopathological features of SIgG4-negative AIP differed from those of SIgG4-positive AIP. Some SIgG4-negative AIP cases are LPSP rather confined to the pancreas. SIgG4-negative AIP may include idiopathic duct-centric pancreatitis (IDCP) or sclerosing pancreatitis other than LPSP or IDCP, but further studies are needed to clarify this issue.

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Acknowledgments

This study was supported by the Research Committee of Intractable Disease, provided by the Ministry of Health, Labour and Welfare of Japan.

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Authors and Affiliations

  1. Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan

    Terumi Kamisawa, Kensuke Takuma, Taku Tabata, Yoshihiko Inaba & Naoto Egawa

  2. Department of Surgery, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    Koji Tsuruta

  3. Department of Pathology, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    Tsunekazu Hishima

  4. Department of Chemotherapy, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    Tsuneo Sasaki

  5. Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan

    Takao Itoi

Authors
  1. Terumi Kamisawa
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  2. Kensuke Takuma
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  3. Taku Tabata
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  4. Yoshihiko Inaba
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  5. Naoto Egawa
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  6. Koji Tsuruta
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  7. Tsunekazu Hishima
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  9. Takao Itoi
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Corresponding author

Correspondence to Terumi Kamisawa.

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Kamisawa, T., Takuma, K., Tabata, T. et al. Serum IgG4-negative autoimmune pancreatitis. J Gastroenterol 46, 108–116 (2011). https://doi.org/10.1007/s00535-010-0317-2

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  • DOI: https://doi.org/10.1007/s00535-010-0317-2

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