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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.
Modern Rheumatology 07/2012; 23(3). DOI:10.1007/s10165-012-0695-9 · 2.21 Impact Factor