International Journal of Rheumatology (Jan 2012)

Cutoff Values of Serum IgG4 and Histopathological IgG4+ Plasma Cells for Diagnosis of Patients with IgG4-Related Disease

  • Yasufumi Masaki,
  • Nozomu Kurose,
  • Motohisa Yamamoto,
  • Hiroki Takahashi,
  • Takako Saeki,
  • Atsushi Azumi,
  • Shinji Nakada,
  • Shoko Matsui,
  • Tomoki Origuchi,
  • Susumu Nishiyama,
  • Kazunori Yamada,
  • Mitsuhiro Kawano,
  • Akira Hirabayashi,
  • Keita Fujikawa,
  • Tomoko Sugiura,
  • Masanobu Horikoshi,
  • Naoto Umeda,
  • Hiroshi Minato,
  • Takuji Nakamura,
  • Haruka Iwao,
  • Akio Nakajima,
  • Miyuki Miki,
  • Tomoyuki Sakai,
  • Toshioki Sawaki,
  • Takafumi Kawanami,
  • Yoshimasa Fujita,
  • Masao Tanaka,
  • Toshihiro Fukushima,
  • Katumi Eguchi,
  • Susumu Sugai,
  • Hisanori Umehara

DOI
https://doi.org/10.1155/2012/580814
Journal volume & issue
Vol. 2012

Abstract

Read online

IgG4-related disease is a new disease classification established in Japan in the 21st century. Patients with IgG4-related disease display hyper-IgG4-gammaglobulinemia, massive infiltration of IgG4+ plasma cells into tissue, and good response to glucocorticoids. Since IgG4 overexpression is also observed in other disorders, it is necessary to diagnose IgG4-related disease carefully and correctly. We therefore sought to determine cutoff values for serum IgG4 and IgG4/IgG and for IgG4+/IgG+ plasma cells in tissue diagnostic of IgG4-related disease. Patients and Methods. We retrospectively analyzed serum IgG4 concentrations and IgG4/IgG ratio and IgG4+/IgG+ plasma cell ratio in tissues of 132 patients with IgG4-related disease and 48 patients with other disorders. Result. Serum IgG4 >135 mg/dl demonstrated a sensitivity of 97.0% and a specificity of 79.6% in diagnosing IgG4-related disease, and serum IgG4/IgG ratios >8% had a sensitivity and specificity of 95.5% and 87.5%, respectively. IgG4+cell/IgG+ cell ratio in tissues >40% had a sensitivity and specificity of 94.4% and 85.7%, respectively. However, the number of IgG4+ cells was reduced in severely fibrotic parts of tissues. Conclusion. Although a recent unanimous consensus of all relevant researchers in Japan recently established the diagnostic criteria for IgG4-related disease, findings such as ours indicate that further discussion is needed.