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NDT Plus Advance Access originally published online on February 23, 2009
NDT Plus 2009 2(3):233-235; doi:10.1093/ndtplus/sfp023
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© The Author [2009]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

A case of tubulointerstitial nephritis in IgG4-related systemic disease with markedly enlarged kidneys

Naobumi Mise1, Yutaka Tomizawa2, Akiko Fujii3, Yutaka Yamaguchi4 and Tokuichiro Sugimoto1

1 Department of Nephrology
2 Department of Internal Medicine
3 Department of Pathology, Mitsui Memorial Hospital, Tokyo, Japan
4 Department of Pathology, Jikei University Kashiwa Hospital, Chiba, Japan

Correspondence: Correspondence and offprint requests to: Naobumi Mise, Department of Nephrology, Mitsui Memorial Hospital, 1 Kanda-izumi-cho, Chiyoda-ku, Tokyo 101-8643, Japan. Tel: +81-3-3862-9111; Fax: +81-3-3862-7765; E-mail: misenn{at}mitsuihosp.or.jp


  Abstract

IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report the case of a 67-year-old man with IgG4-related tubulointerstitial nephritis, presenting with markedly enlarged kidneys and renal dysfunction. The serum IgG4 level was elevated with 4200 mg/dl and pathological examination revealed patchy, clearly fringed areas of IgG4-positive plasma cell infiltration and advanced fibrosis in the renal parenchyma, perirenal tissue and lymph nodes. With oral prednisolone at a dose of 60 mg daily, a contraction of the kidneys and an improvement of renal function were observed. No recurrence of the disease was observed during the reduction of prednisolone to 2 mg daily over 4 years.

Key Words: IgG4 • renal dysfunction • retroperitoneal fibrosis • tubulointerstitial nephritis

Received for publication November 28, 2008. Accepted for publication February 4, 2009.


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