Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis.
IgG4-related disease is a recently recognized multi-organ disorder characterized by high levels of serum IgG4 and dense infiltration of IgG4-positive cells into several organs. Although the pancreas was the first organ recognized to be affected by IgG4-related disorder in the syndrome of autoimmune pancreatitis, we present here clinico-pathological features of 23 patients diagnosed as having renal parenchymal lesions. These injuries were associated with a high level of serum IgG4 and abundant IgG4-positive plasma cell infiltration into the renal interstitium with fibrosis. In all patients, tubulointerstitial nephritis was the major finding. Although 14 of the 23 patients did not have any pancreatic lesions, their clinicopathological features were quite uniform and similar to those shown in autoimmune pancreatitis. These included predominance in middle-aged to elderly men, frequent association with IgG4-related conditions in other organs, high levels of serum IgG and IgG4, a high frequency of hypocomplementemia, a high serum IgE level, a patchy and diffuse lesion distribution, a swirling fibrosis in the renal pathology, and a good response to corticosteroids. Thus, we suggest that renal parenchymal lesions actually develop in association with IgG4-related disease, for which we propose the term 'IgG4-related tubulointerstitial nephritis.'
Saeki
Takako
T
Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Niigata, Japan. saekit@nagaoka.jrc.or.jp
Nishi
Shinichi
S
Imai
Naofumi
N
Ito
Tomoyuki
T
Yamazaki
Hajime
H
Kawano
Mitsuhiro
M
Yamamoto
Motohisa
M
Takahashi
Hiroki
H
Matsui
Shoko
S
Nakada
Shinji
S
Origuchi
Tomoki
T
Hirabayashi
Akira
A
Homma
Noriyuki
N
Tsubata
Yutaka
Y
Takata
Takuma
T
Wada
Yoko
Y
Saito
Akihiko
A
Fukase
Sachiko
S
Ishioka
Kunihiro
K
Miyazaki
Kana
K
Masaki
Yasufumi
Y
Umehara
Hisanori
H
Sugai
Susumu
S
Narita
Ichiei
I
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000936:Antigen-Antibody Complex / Q000378:metabolism; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology; D001706:Biopsy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007073:Immunoglobulin E / Q000378:metabolism; D007074:Immunoglobulin G / Q000378:metabolism*; D007668:Kidney / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D010195:Pancreatitis / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology* / Q000473:pathology*; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies
D000936:Antigen-Antibody Complex; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007073:Immunoglobulin E; D011239:Prednisolone