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

  • Motohisa Yamamoto
  • Tetsuya Tabeya
  • Yasuyoshi Naishiro
  • Hidetaka Yajima
  • Keisuke Ishigami
  • Yui Shimizu
  • Mikiko Obara
  • Chisako Suzuki
  • Kentaro Yamashita
  • Hiroyuki Yamamoto
  • Toshiaki Hayashi
  • Shigeru Sasaki
  • Toshiaki Sugaya
  • Tadao Ishida
  • Ken-ichi Takano
  • Tetsuo Himi
  • Yasuo Suzuki
  • Norihiro Nishimoto
  • Saho Honda
  • Hiroki Takahashi
  • Kohzoh Imai
  • Yasuhisa Shinomura
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

Authors and Affiliations

  • Motohisa Yamamoto
    • 1
  • Tetsuya Tabeya
    • 1
  • Yasuyoshi Naishiro
    • 1
  • Hidetaka Yajima
    • 1
  • Keisuke Ishigami
    • 1
  • Yui Shimizu
    • 1
  • Mikiko Obara
    • 1
  • Chisako Suzuki
    • 1
  • Kentaro Yamashita
    • 1
  • Hiroyuki Yamamoto
    • 1
  • Toshiaki Hayashi
    • 1
  • Shigeru Sasaki
    • 1
  • Toshiaki Sugaya
    • 1
  • Tadao Ishida
    • 1
  • Ken-ichi Takano
    • 2
  • Tetsuo Himi
    • 2
  • Yasuo Suzuki
    • 3
  • Norihiro Nishimoto
    • 4
  • Saho Honda
    • 5
  • Hiroki Takahashi
    • 1
  • Kohzoh Imai
    • 1
    • 6
  • Yasuhisa Shinomura
    • 1
  1. 1.First Department of Internal MedicineSapporo Medical University School of MedicineSapporoJapan
  2. 2.Department of OtolaryngologySapporo Medical University School of MedicineSapporoJapan
  3. 3.Department of OphthalmologyTeine Keijinkai HospitalSapporoJapan
  4. 4.Laboratory of Immune RegulationWakayama Medical UniversityOsakaJapan
  5. 5.Department of Internal Medicine and RheumatologyJR Sapporo HospitalSapporoJapan
  6. 6.Advanced Clinical Research Center, The Institute of Medical ScienceThe University of TokyoTokyoJapan