JOURNAL ARTICLE

Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis

Takako Saeki, Shinichi Nishi, Naofumi Imai, Tomoyuki Ito, Hajime Yamazaki, Mitsuhiro Kawano, Motohisa Yamamoto, Hiroki Takahashi, Shoko Matsui, Shinji Nakada, Tomoki Origuchi, Akira Hirabayashi, Noriyuki Homma, Yutaka Tsubata, Takuma Takata, Yoko Wada, Akihiko Saito, Sachiko Fukase, Kunihiro Ishioka, Kana Miyazaki, Yasufumi Masaki, Hisanori Umehara, Susumu Sugai, Ichiei Narita
Kidney International 2010, 78 (10): 1016-23
20720530
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.'

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