Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Niigata, Japan.
Kidney Int. 2010 Nov;78(10):1016-23. doi: 10.1038/ki.2010.271. Epub 2010 Aug 18.
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.'
IgG4 相关疾病是一种新近被认识的多器官疾病，其特征为血清 IgG4 水平升高和 IgG4 阳性细胞在多个器官中的密集浸润。虽然胰腺是自身免疫性胰腺炎综合征中最早被认识到受 IgG4 相关疾病影响的器官，但我们在此报告了 23 例被诊断为肾实质病变的患者的临床病理特征。这些损伤与血清 IgG4 水平升高和 IgG4 阳性浆细胞在肾间质伴纤维化中的丰富浸润有关。在所有患者中，肾小管间质性肾炎是主要发现。尽管 23 例患者中有 14 例没有任何胰腺病变，但他们的临床病理特征相当一致，类似于自身免疫性胰腺炎。这些特征包括好发于中老年男性、常与其他器官的 IgG4 相关疾病相关、血清 IgG 和 IgG4 水平升高、补体血症频繁、血清 IgE 水平升高、斑片状和弥漫性病变分布、肾病理中的漩涡状纤维化以及对皮质类固醇的良好反应。因此，我们建议肾实质病变实际上是与 IgG4 相关疾病相关的，我们提出了“IgG4 相关肾小管间质性肾炎”这一术语。
