Vol.22 No.3

Original Article

Risk of malignancies in IgG4-related disease

Authors

Motohisa Yamamoto1 , Hiroki Takahashi1 , Tetsuya Tabeya1 , Chisako Suzuki1 , Yasuyoshi Naishiro1 , Keisuke Ishigami1 , Hidetaka Yajima1 , Yui Shimizu1 , Mikiko Obara1 , Hiroyuki Yamamoto1 , Tetsuo Himi2 , Kohzoh Imai3, Yasuhisa Shinomura1

  • First Department of Internal Medicine, Sapporo Medical University School of Medicine, South 1-West 16, Chuo-ku, Sapporo, Hokkaido, 0608543, Japan
  • Department of Otolaryngology, Sapporo Medical University School of Medicine, Sapporo, Japan
  • Advanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
Received:

27 July 2011

Accepted:

16 August 2011

Published online:

6 September 2011

Full Text

PDF　(member's only)

Abstract

IgG4-related disease (IgG4-RD) is considered a systemic, chronic, and inflammatory disorder that is characterized by the enlargement of involved organs, elevated levels of IgG4, and abundant infiltration of plasmacytes with IgG4 and fibrosis in involved organs. It is necessary to differentiate IgG4-RD from malignant tumors. Recently we have looked at case reports of IgG4-RD with malignancy that was discovered at systemic screening. In this study, we analyzed the relationship between IgG4-RD and malignancies. The study subjects were 106 patients with IgG4-RD who had been referred to our hospital since April 1997. We analyzed the clinical characteristics of IgG4-RD patients who had cancer that was observed upon the initial diagnosis of IgG4-RD or that occurred during an average follow-up period of 3.1 years. Using data from national cancer registries that monitor cancer incidence in Japan, we evaluated the standardized incidence ratio (SIR) for malignancies in IgG4-RD. Malignancies were observed in 11 of the IgG4-RD patients (10.4%). The malignancies were all different and included lung cancer, colon cancer, and lymphoma. With the exception of the age at which the IgG4-RD diagnosis was made, there were no common features in patients with cancer and those without. The SIR for these malignancies in IgG4-RD was 383.0, which was higher than that for the general population. We should be cognizant of the possible existence of malignancies in patients with IgG4-RD at the time of diagnosis and during follow-up care.

Key words

Cancer - IgG4 - Lymphoma