Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis
Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis
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DOI：
10.1038/ki.2010.271
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发表时间：
2010-11-01
影响因子：
19.6
通讯作者：
Narita, Ichiei
中科院分区：
文献类型：
--
作者：
Saeki, Takako;Nishi, Shinichi;Narita, Ichiei
IgG4相关疾病是一种新近发现的多器官疾病，其特征为血清IgG4水平高和IgG4阳性细胞密集浸润到多个器官中。虽然胰腺是第一个被认为是受IgG 4相关疾病的自身免疫性胰腺炎综合征的器官，我们在这里提出了23例诊断为肾实质病变的临床病理特征。这些损伤与高水平的血清IgG 4和大量IgG 4阳性浆细胞浸润到肾间质纤维化有关。在所有患者中，肾小管间质性肾炎是主要发现。虽然23例患者中有14例没有任何胰腺病变，但其临床病理特征非常一致，与自身免疫性胰腺炎相似。这些包括中年至老年男性的优势，其他器官中与IgG 4相关疾病的频繁关联，高水平的血清IgG和IgG 4，低补体血症的高频率，高血清IgE水平，斑片状和弥漫性病变分布，肾脏病理学中的旋转纤维化，以及对皮质类固醇的良好反应。因此，我们认为肾实质病变实际上与IgG 4相关疾病有关，我们提出术语“IgG 4相关肾小管间质性肾炎”。'
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 clinicopathological 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.'