Vol.23 No.3

Case Report

A case of Epstein-Barr virus-related lymphadenopathy mimicking the clinical features of IgG4-related disease

Authors

Yoko Wada1 , Masaru Kojima2 , Kazuhiro Yoshita1 , Mihoko Yamazaki1 , Daisuke Kobayashi1 , Shuichi Murakami1 , Shinichi Nishi3 , Masaaki Nakano4 , Ichiei Narita1

  • Division of Clinical Nephrology and Rheumatology, Department of Medicine II, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Chuo-ku, Niigata Niigata, 951-8510, Japan
  • Anatomic and Diagnostic Pathology, Dokkyo Medical University, Tochigi, Japan
  • Division of Nephrology and Kidney Center, Kobe University Graduate School of Medicine, Kobe, Japan
  • Department of Medical Technology, School of Health Sciences, Faculty of Medicine, Niigata University, Niigata, Japan
Received:

14 March 2012

Accepted:

30 May 2012

Published online:

28 July 2012

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Abstract

We report an intriguing case of Epstein? Barr virus (EBV)-related multiple lymphadenopathy that clinically mimics immunoglobulin G4-related disease (IgG4-RD). A 72-year-old woman presented with a history of asthma attacks, systemic lymphadenopathy, hypergammaglobulinemia, proteinuria, and an elevated level of serum IgG4, leading to a possible diagnosis of IgG4-RD based on current comprehensive diagnostic criteria. However, a percutaneous kidney biopsy specimen showed mild mesangial proliferative glomerulonephritis with focal membranous transformation, and there was no interstitial lesion or lymphocyte infiltration. Cervical lymph node biopsy demonstrated follicular hyperplasia associated with prominent lymphoplasmacytic infiltration in the interfollicular area. However, only a few IgG4-positive plasma cells were present. An in situ hybridization study demonstrated many EBV-infected lymphocytes in the germinal center as well as in the interfollicular area. This case illustrates the diversity of conditions associated with elevated levels of serum IgG4 and the necessity for tissue biopsy when diagnosing IgG4-RD.

Key words

Epstein-Barr virus, Systemic lymphadenopathy, Bronchial asthma, Membranous nephropathy, IgG4-related disease