Multicentric castleman’s disease mimicking IgG4-related disease

A case report

Eiko Hasegawa, Akinari Sekine, Jun Ichi Inenaga, Takeshi Fujii, Kenichi Ohashi, Yasuharu Sato, Yoshifumi Ubara

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

A 63-year-old Japanese man was admitted to our hospital for the evaluation of hypergammaglobulinemia, lymphadenopathy, interstitial lung disease and nephritis. His serum level of IgG and IgG4 were 8198 mg/dL and 3360 mg/dL, respectively. Renal biopsy revealed focal interstitial nephritis with IgG4 positive plasma cells, with an IgG4/IgG ratio of >0.6. Inguinal lymph node biopsy showed hyperplasia of the lymphoid follicles with prominent infiltration of mature plasma cells between normal germinal centers. Immunostaining showed that the IgG4-positive plasma cells were prominent, and the IgG4/IgG ratio within the lymph node was >0.8. Although these findings were consistent with IgG4-related disease (IgG4-RD), other laboratory findings such as high serum concentrations of C-reactive protein, interleukin-6, IgA, and IgM, as well as positive staining of the lymph node for IgA and IL-6, suggested multicentric Castleman’s disease (MCD). Following unsuccessful therapy with glucocorticoids, treatment with an anti-interleukin-6 receptor monoclonal antibody (tocilizumab) exerted disease control. Understanding the crucial differences between MCD and IgG4-RD may contribute to understanding the pathogenesis of both diseases.

Original languageEnglish
Title of host publicationIgG4-Related Kidney Disease
PublisherSpringer Japan
Pages293-301
Number of pages9
ISBN (Electronic)9784431556879
ISBN (Print)9784431556862
DOIs
Publication statusPublished - Jan 1 2016

Fingerprint

Immunoglobulin G
Plasma Cells
Lymph Nodes
Immunoglobulin A
Interleukin-6
Multi-centric Castleman's Disease
Hypergammaglobulinemia
Biopsy
Interleukin-6 Receptors
Interstitial Nephritis
Germinal Center
Groin
Nephritis
Interstitial Lung Diseases
Serum
C-Reactive Protein
Glucocorticoids
Hyperplasia
Immunoglobulin M
Monoclonal Antibodies

Keywords

  • IgG4-related disease
  • IL-6
  • Multicentric castleman’s disease

ASJC Scopus subject areas

  • Medicine(all)
  • Immunology and Microbiology(all)

Cite this

Hasegawa, E., Sekine, A., Inenaga, J. I., Fujii, T., Ohashi, K., Sato, Y., & Ubara, Y. (2016). Multicentric castleman’s disease mimicking IgG4-related disease: A case report. In IgG4-Related Kidney Disease (pp. 293-301). Springer Japan. https://doi.org/10.1007/978-4-431-55687-9_26

Multicentric castleman’s disease mimicking IgG4-related disease : A case report. / Hasegawa, Eiko; Sekine, Akinari; Inenaga, Jun Ichi; Fujii, Takeshi; Ohashi, Kenichi; Sato, Yasuharu; Ubara, Yoshifumi.

IgG4-Related Kidney Disease. Springer Japan, 2016. p. 293-301.

Research output: Chapter in Book/Report/Conference proceedingChapter

Hasegawa, E, Sekine, A, Inenaga, JI, Fujii, T, Ohashi, K, Sato, Y & Ubara, Y 2016, Multicentric castleman’s disease mimicking IgG4-related disease: A case report. in IgG4-Related Kidney Disease. Springer Japan, pp. 293-301. https://doi.org/10.1007/978-4-431-55687-9_26
Hasegawa E, Sekine A, Inenaga JI, Fujii T, Ohashi K, Sato Y et al. Multicentric castleman’s disease mimicking IgG4-related disease: A case report. In IgG4-Related Kidney Disease. Springer Japan. 2016. p. 293-301 https://doi.org/10.1007/978-4-431-55687-9_26
Hasegawa, Eiko ; Sekine, Akinari ; Inenaga, Jun Ichi ; Fujii, Takeshi ; Ohashi, Kenichi ; Sato, Yasuharu ; Ubara, Yoshifumi. / Multicentric castleman’s disease mimicking IgG4-related disease : A case report. IgG4-Related Kidney Disease. Springer Japan, 2016. pp. 293-301
@inbook{db05a439d2e54d21b99adb32fc4e2177,
title = "Multicentric castleman’s disease mimicking IgG4-related disease: A case report",
abstract = "A 63-year-old Japanese man was admitted to our hospital for the evaluation of hypergammaglobulinemia, lymphadenopathy, interstitial lung disease and nephritis. His serum level of IgG and IgG4 were 8198 mg/dL and 3360 mg/dL, respectively. Renal biopsy revealed focal interstitial nephritis with IgG4 positive plasma cells, with an IgG4/IgG ratio of >0.6. Inguinal lymph node biopsy showed hyperplasia of the lymphoid follicles with prominent infiltration of mature plasma cells between normal germinal centers. Immunostaining showed that the IgG4-positive plasma cells were prominent, and the IgG4/IgG ratio within the lymph node was >0.8. Although these findings were consistent with IgG4-related disease (IgG4-RD), other laboratory findings such as high serum concentrations of C-reactive protein, interleukin-6, IgA, and IgM, as well as positive staining of the lymph node for IgA and IL-6, suggested multicentric Castleman’s disease (MCD). Following unsuccessful therapy with glucocorticoids, treatment with an anti-interleukin-6 receptor monoclonal antibody (tocilizumab) exerted disease control. Understanding the crucial differences between MCD and IgG4-RD may contribute to understanding the pathogenesis of both diseases.",
keywords = "IgG4-related disease, IL-6, Multicentric castleman’s disease",
author = "Eiko Hasegawa and Akinari Sekine and Inenaga, {Jun Ichi} and Takeshi Fujii and Kenichi Ohashi and Yasuharu Sato and Yoshifumi Ubara",
year = "2016",
month = "1",
day = "1",
doi = "10.1007/978-4-431-55687-9_26",
language = "English",
isbn = "9784431556862",
pages = "293--301",
booktitle = "IgG4-Related Kidney Disease",
publisher = "Springer Japan",

}

TY - CHAP

T1 - Multicentric castleman’s disease mimicking IgG4-related disease

T2 - A case report

AU - Hasegawa, Eiko

AU - Sekine, Akinari

AU - Inenaga, Jun Ichi

AU - Fujii, Takeshi

AU - Ohashi, Kenichi

AU - Sato, Yasuharu

AU - Ubara, Yoshifumi

PY - 2016/1/1

Y1 - 2016/1/1

N2 - A 63-year-old Japanese man was admitted to our hospital for the evaluation of hypergammaglobulinemia, lymphadenopathy, interstitial lung disease and nephritis. His serum level of IgG and IgG4 were 8198 mg/dL and 3360 mg/dL, respectively. Renal biopsy revealed focal interstitial nephritis with IgG4 positive plasma cells, with an IgG4/IgG ratio of >0.6. Inguinal lymph node biopsy showed hyperplasia of the lymphoid follicles with prominent infiltration of mature plasma cells between normal germinal centers. Immunostaining showed that the IgG4-positive plasma cells were prominent, and the IgG4/IgG ratio within the lymph node was >0.8. Although these findings were consistent with IgG4-related disease (IgG4-RD), other laboratory findings such as high serum concentrations of C-reactive protein, interleukin-6, IgA, and IgM, as well as positive staining of the lymph node for IgA and IL-6, suggested multicentric Castleman’s disease (MCD). Following unsuccessful therapy with glucocorticoids, treatment with an anti-interleukin-6 receptor monoclonal antibody (tocilizumab) exerted disease control. Understanding the crucial differences between MCD and IgG4-RD may contribute to understanding the pathogenesis of both diseases.

AB - A 63-year-old Japanese man was admitted to our hospital for the evaluation of hypergammaglobulinemia, lymphadenopathy, interstitial lung disease and nephritis. His serum level of IgG and IgG4 were 8198 mg/dL and 3360 mg/dL, respectively. Renal biopsy revealed focal interstitial nephritis with IgG4 positive plasma cells, with an IgG4/IgG ratio of >0.6. Inguinal lymph node biopsy showed hyperplasia of the lymphoid follicles with prominent infiltration of mature plasma cells between normal germinal centers. Immunostaining showed that the IgG4-positive plasma cells were prominent, and the IgG4/IgG ratio within the lymph node was >0.8. Although these findings were consistent with IgG4-related disease (IgG4-RD), other laboratory findings such as high serum concentrations of C-reactive protein, interleukin-6, IgA, and IgM, as well as positive staining of the lymph node for IgA and IL-6, suggested multicentric Castleman’s disease (MCD). Following unsuccessful therapy with glucocorticoids, treatment with an anti-interleukin-6 receptor monoclonal antibody (tocilizumab) exerted disease control. Understanding the crucial differences between MCD and IgG4-RD may contribute to understanding the pathogenesis of both diseases.

KW - IgG4-related disease

KW - IL-6

KW - Multicentric castleman’s disease

UR - http://www.scopus.com/inward/record.url?scp=85018534012&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85018534012&partnerID=8YFLogxK

U2 - 10.1007/978-4-431-55687-9_26

DO - 10.1007/978-4-431-55687-9_26

M3 - Chapter

SN - 9784431556862

SP - 293

EP - 301

BT - IgG4-Related Kidney Disease

PB - Springer Japan

ER -