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Autoimmunity
Original article
Distinct features distinguishing IgG4-related disease from multicentric Castleman's disease
  1. Takanori Sasaki1,
  2. Mitsuhiro Akiyama1,
  3. Yuko Kaneko1,
  4. Takehiko Mori2,
  5. Hidekata Yasuoka1,
  6. Katsuya Suzuki1,
  7. Kunihiro Yamaoka1,
  8. Shinichiro Okamoto2 and
  9. Tsutomu Takeuchi1
  1. 1 Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
  2. 2 Division of Hematology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
  1. Correspondence to Dr Tsutomu Takeuchi; tsutake{at}z5.keio.jp

Abstract

Objectives Differentiating IgG4-related disease (IgG4-RD) from multicentric Castleman’s disease (MCD) is challenging because both diseases present high serum IgG4. The objective of this study is to clarify the differences in characteristics and identify a clinically useful approach to differentiate these two diseases.

Methods Forty-five consecutive patients with untreated active IgG4-RD and 33 patients with MCD were included in this study, who visited our institution from January 2000 to August 2016. The clinical and laboratory findings for the patients of the two diseases were compared. Various combinations of the distinctive findings were evaluated to identify the most efficient differentiating features between IgG4-RD and MCD.

Results The levels of serum IgG4 were not different between the two diseases. Orbits, lacrimal glands, salivary glands or pancreas were involved in 88.9% of IgG4-RD cases and only in 3.0% of MCD cases. All MCD cases involved lymph nodes. Atopic history was characteristic for IgG4-RD. The levels of C reactive protein (CRP) with a cut-off of 0.80 mg/dL and IgA with a cut-off of 330 mg/dL were the most distinctive. The combination of ‘Orbits, lacrimal glands, salivary glands or pancreas involvement, atopic history, or non-involvement of lymph node’ and ‘CRP ≤ 0.8 mg/dL or IgA ≤ 330 mg/dL’ yielded the probability of 97.8% in IgG4-RD, while that of 3.0 % in patients with MCD.

Conclusions Our study revealed distinct features between IgG4-RD and MCD. Differentiating between the diseases based on those distinct features, including distribution of organ involvement, atopic history, levels of IgA and CRP, was a useful approach.

  • Autoimmune diseases
  • Autoimmunity
  • Inflammation

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/rmdopen-2017-000432

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    Footnotes

    • Contributors TS and MA participated in the study design, reviewed the patient data, performed the statistical analysis, evaluated the results and drafted the manuscript. YK, TM, HY, KS and KY participated in the study design and helped to draft the manuscript. SO and TT conceived the study, participated in its design and coordination, evaluated the results and helped to draft the manuscript. All authors have read and approved the final version of the manuscript.

    • Competing interests TS has received consultancies, speaking fees and honoraria from Eisai Co Ltd. MA has received consultancies, speaking fees and honoraria from Cure Grades Co and Eisai Co Ltd. YK has received consultancies, speaking fees and honoraria from AbbVie GK, Astellas Pharma, Bristol–Myers KK, Chugai Pharmaceutical Co Ltd, Eisai Co, Ltd, Janssen Pharmaceutical KK, Kyowa Hakko Kirin Co Ltd, Mitsubishi Tanabe Pharma Co, Pfizer Japan Inc, Santen Pharmaceutical Co, Taisho Toyama Pharma Co and UCB. TM has received consulting fees, speaking fees and honoraria from Astellas Pharma, Chugai Pharmaceutical, Eisai and Janssen Pharmaceutical KK. HY declares no competing interest. KS has received consulting fees, speaking fees and honoraria from Bristol–Myers Squibb Company, Daichi Sankyo Co Ltd, Eisai Co Ltd and Kissei Pharmaceutical Co Ltd. KY has received consultancies, speaking fees and honoraria from AbbVie GK, Acterlion Pharmaceuticals, Astellas Pharma, Bristol–Myers KK, Chugai Pharmaceutical Co Ltd, Eisai Co Ltd, Eli Lilly Japan KK, GlaxoSmithkline, Janssen Pharmaceutical KK, Mitsubishi Tanabe Pharma Co, Nippon Shinyaku Co Ltd, Pfizer Japan Inc and Takeda Pharmaceutical Co Ltd. SO has received consulting fees, speaking fees and honoraria from Astellas Pharma, Bristol-Myers KK, Chugai Pharmaceutical, Eisai, Pfizer Japan and Takeda Pharmaceutical. TT has received consultancies, speaking fees and honoraria from AbbVie GK, Asahikasei Pharma Corp, Astellas Pharma, Bristol–Myers KK, Chugai Pharmaceutical Co Ltd, Eisai Co Ltd, Janssen Pharmaceutical KK, Mitsubishi Tanabe Pharma Co, Pfizer Japan Inc, Takeda Pharmaceutical Co Ltd and UCB.

    • Ethics approval The ethics committee at Keio University School of Medicine.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data sharing statement No additional data are available.