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  • Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castleman disease (iMCD): Factors to differentiate from IgG4-related disease

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Original research
Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castleman disease (iMCD): Factors to differentiate from IgG4-related disease
  1. Asami Nishikori1,2,
  2. Midori Filiz Nishimura1,2,
  3. David C Fajgenbaum3,4,
  4. Yoshito Nishimura5,6,
  5. Kanna Maehama1,
  6. Tomoka Haratake1,
  7. Tetsuya Tabata7,
  8. Mitsuhiro Kawano2,8,
  9. Naoya Nakamura9,
  10. Shuji Momose10,
  11. Remi Sumiyoshi2,11,
  12. Tomohiro Koga2,11,
  13. Hidetaka Yamamoto12,
  14. Frits van Rhee13,
  15. Atsushi Kawakami2,11,
  16. Yasuharu Sato1,2
  1. 1Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama, Japan
  2. 2Research Program for Intractable Disease by Ministry of Health, Tokyo, Japan
  3. 3Department of Medicine, Center for Cytokine Storm Treatment & Laboratory, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
  4. 4Castleman Disease Collaborative Network, Philadelphia, Pennsylvania, USA
  5. 5Department of General Medicine, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan
  6. 6Department of Medicine, University of Hawai’i, Honolulu, Hawaiʻi, USA
  7. 7Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan
  8. 8Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
  9. 9Department of Pathology, Tokai University School of Medicine, Isehara, Japan
  10. 10Department of Pathology, Saitama Medical University, Saitama, Japan
  11. 11Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
  12. 12Department of Pathology and Oncology, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan
  13. 13University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
  1. Correspondence to Dr Yasuharu Sato, Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama, Japan; satou-y{at}okayama-u.ac.jp

Abstract

Aims and methods Idiopathic multicentric Castleman disease (iMCD) is currently considered to be classified into three clinical subtypes, including idiopathic plasmacytic lymphadenopathy (IPL), thrombocytopaenia, anasarca, fever, reticulin fibrosis/renal dysfunction, organomegaly (TAFRO) and not otherwise specified (NOS). Among the three, iMCD-IPL closely mimics IgG4-related disease (IgG4-RD). In diagnosing IgG4-RD, it is sometimes challenging to distinguish iMCD-IPL patients that also meet the histological diagnostic criteria for IgG4-RD. In this study, we focused on the number of IgG4-positive cells in the lymph nodes and analysed the relationship with laboratory findings to distinguish iMCD-IPL from IgG4-RD. Thirty-nine patients with iMCD-IPL and 22 patients with IgG4-RD were included.

Results Among the cases considered to be iMCD-IPL, 33.3% (13/39) cases also met the histological diagnostic criteria for IgG4-RD and serum IgG4 levels were not different between the two groups. However, the serum IgG4/IgG ratio was significantly higher in IgG4-RD, with a cut-off value of 19.0%. Additionally, a significant positive correlation between serum IgG levels and the number of IgG4-positive cells was observed in iMCD-IPL (p=0.001). The serum IgG cut-off value for distinguishing iMCD-IPL meeting histological criteria for IgG4-RD from other iMCD-IPL was 5381 mg/dL.

Conclusions iMCD-IPL cases with high serum IgG levels (>5000 mg/dL) were likely to meet the diagnostic criteria for IgG4-RD because of the numerous IgG4-positive cells observed. A combination of clinical presentations, laboratory values including the serum IgG4/IgG ratios and histological analysis is crucial for diagnosis of IgG4-RD and iMCD-IPL.

  • Hematology
  • Histology
  • IMMUNOHISTOCHEMISTRY
  • INFLAMMATION
  • LYMPH NODES

Data availability statement

Data sharing not applicable as no datasets generated and/or analysed for this study. Not applicable.

https://doi.org/10.1136/jcp-2023-209280

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    Data availability statement

    Data sharing not applicable as no datasets generated and/or analysed for this study. Not applicable.

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    Footnotes

    • Handling editor Vikram Deshpande.

    • AN and MFN contributed equally.

    • Contributors Conceptualisation, AN, MFN and YS; methodology, AN; formal analysis, AN; investigation, AN, KM, TT, MK, NN, SM, RS, TK, AK and HY; data curation, AN, KM and TH; writing—original draft preparation, AN; writing—review and editing, YN, DCF and FvR; supervision, FvR and YS. YS is responsible for the overall content as guarantor. All authors have read and agreed to the published version of the manuscript.

    • Funding This work was supported by JSPS KAKENHI grant number JP 23K1447605, MHLW Programme grant number JPMH 23FC1025, JPMH 23FC1015 and AMED under grant number JP 22ek0109589h0001.

    • Competing interests None declared.

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