A Case of Immunoglobulin G4-related Interstitial Nephritis with Bicytopenia

Soonkyu Lee; Yune-Jung Park; Uiju Cho; Young Jin Choi; Wan-Uk Kim

doi:10.4078/jrd.2015.22.6.401

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Lee, Park, Cho, Choi, and Kim: A Case of Immunoglobulin G4-related Interstitial Nephritis with Bicytopenia

Case Report

J Rheum Dis 2015;22(6):401-404.

Published online: 31 October 2015

DOI: https://doi.org/10.4078/jrd.2015.22.6.401

A Case of Immunoglobulin G4-related Interstitial Nephritis with Bicytopenia

Soonkyu Lee¹, Yune-Jung Park², Uiju Cho³, Young Jin Choi³, Wan-Uk Kim⁴

¹Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea

²Division of Rheumatology, Department of Internal Medicine, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Suwon, Korea

³Department of Hospital Pathology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

⁴Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

Corresponding to: Wan-Uk Kim, Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 06591, Korea. E-mail: wan725@catholic.ac.kr

Received 29 December 2014 Revised 27 March 2015 Revised 7 April 2015 Accepted 7 April 2015

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease found in many organs including biliary tract, salivary gland, kidney, and lung. Tubulointerstitial nephritis is the most common renal manifestation, but hematologic involvement of IgG4-RD is rare. Here, we report on a case of a 57-year-old male with IgG4-related interstitial nephritis with bicytopenia, which was initially thought to be systemic lupus erythematosus. He presented with proteinuria, anemia, thrombocytopenia, and low complement levels. Histological findings showed an increased number of IgG4-positive plasma cells (＞200/high power field), and an elevated IgG4/IgG ratio (＞90%). Serum levels of IgG and IgG4 were also increased. This case emphasized the importance of differential diagnosis of IgG4-RD and immune complex glomerulonephritis.

Keywords: Immunoglobulin G4-related disease, Thrombocytopenia, Anemia, Interstitial nephritis, Hypocomplementemia

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Figure 1.

(A) Renal biopsy shows diffuse infiltration of inflammatory cells and stromal fibrosis, which are replacing the normal renal parenchyma. As a result, renal tubules are severely atrophied and some glomeruli show global glomerulosclerosis (H&E, ×200). (B) On higher magnification, infiltrating inflammatory cells are composed of many lymphocytes, plasma cells and a few eosinophils (H&E, ×400). (C) Immunohistochemical staining reveals that the plasma cells are mostly immunoglobulin G4 (IgG4) positive. There are more than 200 IgG4 positive plasma cells in a high power field and more than 90% of IgG positive cells express IgG4 (IgG immunostaining, ×200).

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