Modern Rheumatology

, Volume 22, Issue 3, pp 419–425

Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases

Authors

    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Tetsuya Tabeya
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Yasuyoshi Naishiro
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Hidetaka Yajima
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Keisuke Ishigami
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Yui Shimizu
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Mikiko Obara
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Chisako Suzuki
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Kentaro Yamashita
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Hiroyuki Yamamoto
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Toshiaki Hayashi
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Shigeru Sasaki
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Toshiaki Sugaya
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Tadao Ishida
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Ken-ichi Takano
    • Department of OtolaryngologySapporo Medical University School of Medicine
  • Tetsuo Himi
    • Department of OtolaryngologySapporo Medical University School of Medicine
  • Yasuo Suzuki
    • Department of OphthalmologyTeine Keijinkai Hospital
  • Norihiro Nishimoto
    • Laboratory of Immune RegulationWakayama Medical University
  • Saho Honda
    • Department of Internal Medicine and RheumatologyJR Sapporo Hospital
  • Hiroki Takahashi
    • First Department of Internal MedicineSapporo Medical University School of Medicine
  • Kohzoh Imai
    • First Department of Internal MedicineSapporo Medical University School of Medicine
    • Advanced Clinical Research Center, The Institute of Medical ScienceThe University of Tokyo
  • Yasuhisa Shinomura
    • First Department of Internal MedicineSapporo Medical University School of Medicine
Original Article

DOI: 10.1007/s10165-011-0532-6

Cite this article as:
Yamamoto, M., Tabeya, T., Naishiro, Y. et al. Mod Rheumatol (2012) 22: 419. doi:10.1007/s10165-011-0532-6

Abstract

IgG4-related disease (IgG4-RD) is a novel disease entity that includes Mikulicz’s disease, autoimmune pancreatitis (AIP), and many other conditions. It is characterized by elevated serum IgG4 levels and abundant IgG4-bearing plasmacyte infiltration of involved organs. We postulated that high levels of serum IgG4 would comprise a useful diagnostic tool, but little information is available about IgG4 in conditions other than IgG4-RD, including rheumatic diseases. Several reports have described cutoff values for serum IgG4 when diagnosing IgG4-RD, but these studies mostly used 135 mg/dL in AIP to differentiate from pancreatic cancer instead of rheumatic and other common diseases. There is no evidence for a cutoff serum IgG4 level of 135 mg/dL for rheumatic diseases and common diseases that are often complicated with rheumatic diseases. The aim of this work was to re-evaluate the usual cutoff serum IgG4 value in AIP (135 mg/dL) that is used to diagnose whole IgG4-RD in the setting of a rheumatic clinic by measuring serum IgG4 levels in IgG4-RD and various disorders. We therefore constructed ROC curves of serum IgG4 levels in 418 patients who attended Sapporo Medical University Hospital due to IgG4-RD and various rheumatic and common disorders. The optimal cut-off value of serum IgG4 for a diagnosis of IgG4-RD was 144 mg/dL, and the sensitivity and specificity were 95.10 and 90.76%, respectively. Levels of serum IgG4 were elevated in IgG4-RD, Churg–Strauss syndrome, multicentric Castleman’s disease, eosinophilic disorders, and in some patients with rheumatoid arthritis, systemic sclerosis, chronic hepatitis, and liver cirrhosis. The usual cut-off value of 135 mg/dL in AIP is useful for diagnosing whole IgG4-RD, but high levels of serum IgG4 are sometimes observed in not only IgG4-RD but also other rheumatic and common diseases.

Keywords

Autoimmune pancreatitisChurg–Strauss syndromeIgG4Mikulicz’s diseaseRheumatoid arthritis

Copyright information

© Japan College of Rheumatology 2011