The Korean Journal of Internal Medicine

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Case Report
Korean J Med. 2014;87(1):96-100. Published online July 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.87.1.96
헤노흐-쇤라인 자색반과 동반된 IgG4 연관 요세관간질 신염
양 현, 최수경, 김보경, 유지연, 고은실, 장윤식, 정성진
가톨릭대학교 의과대학 내과학교실
IgG4-Related Tubulointerstitial Nephritis Accompanied by Henoch-Schönlein Purpura
Hyun Yang, Soo Kyoung Choi, Bokyoung Kim, Ji Yeon Yoo, Eun Sil Koh, Yoon Sik Chang, Sungjin Chung
Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea
Corresponding author: Sungjin Chung ,Tel: +82-2-3779-2413, Fax: +82-2-780-3132, Email: sungjin.chung@outlook.com
Received: September 13, 2013; Revised: October 23, 2013   Accepted: November 7, 2013.


Abstract
Tubulointerstitial nephritis is one of the common manifestations of immunoglobulin G (IgG)4-related disease; however, among all cases of tubulointerstitial nephritis undergoing renal biopsies, IgG4-related tubulointerstitial nephritis seems to be relatively rare because of its trivial urinary findings. A previously healthy 54-year-old man was referred to our clinic with a 4-week history of lower leg purpura and renal dysfunction. A kidney biopsy was planned because of bilateral renomegaly, by imaging studies, and elevated serum creatinine levels. Pathological findings in the kidney showed prominent infiltration of IgG4-postive plasma cells in the tubulointerstitium, but not the glumeruli. A skin biopsy revealed leukocytoclastic vasculitis, accompanied by deposition of IgA and C3 in the vascular wall, indicating Henoch-Schönlein purpura (HSP). Although cases of combined IgG4-related disease and microvasculitis, including HSP, are extremely rare, the possibility of an association between two diseases deserves attention.

Keywords :Immunoglobulin G4, Nephritis, Interstitial, Henoch-Schönlein purpura

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