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Case Reports
. 2016 Sep;26(5):784-9.
doi: 10.3109/14397595.2014.916836.

A condition closely mimicking IgG4-related disease despite the absence of serum IgG4 elevation and IgG4-positive plasma cell infiltration

Satoshi Hara  1   2 , Mitsuhiro Kawano  1 , Ichiro Mizushima  1 , Kazunori Yamada  1 , Kentaro Fujita  3 , Kenichi Harada  4 , Masami Matsumura  5 , Masakazu Yamagishi  6 , Yasuharu Sato  7 , Yutaka Yamaguchi  8 , Yasuni Nakanuma  4 , Michio Nagata  2
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Free article
Case Reports

A condition closely mimicking IgG4-related disease despite the absence of serum IgG4 elevation and IgG4-positive plasma cell infiltration

Satoshi Hara et al. Mod Rheumatol. 2016 Sep.
Free article

Abstract

We describe a 74-year-old Japanese man with systemic fibroinflammatory conditions closely resembling those of immunoglobulin G4-related disease (IgG4-RD). Radiology and histology showed characteristics of IgG4-related tubulointerstitial nephritis, despite normal serum IgG4 value and scanty IgG4-positive plasma cell infiltration in each organ. This case suggests that a condition closely mimicking IgG4-RD may develop without IgG4-positive plasma cells and those exceptional cases should also be taken into account in the differential diagnosis of IgG4-RD.

Keywords: IgG4; IgG4-related disease; Tubulointerstitial nephritis.

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