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中华肾病研究电子杂志 ›› 2013, Vol. 02 ›› Issue (01) : 28 -33. doi: 10.3877/cma.j.issn.2095-3216.2013.01.006

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IgG4相关肾病的诊断与治疗
孙晶娣1,2, 李文歌1,(), 赵久阳2   
  1. 1.100029 北京,卫生部中日友好医院肾内科
    2.大连医科大学附属第二医院肾内科
  • 出版日期:2013-02-15
  • 通信作者: 李文歌

The diagnosis and treatment of the IgG4-related kidney disease

Jing-di SUN1, Wen-ge LI1,(), Jiu-yang ZHAO1   

  1. 1.Department of Nephrology, China-Japan Friendship Hospital, Beijing 100029,China
  • Published:2013-02-15
  • Corresponding author: Wen-ge LI
引用本文:

孙晶娣, 李文歌, 赵久阳. IgG4相关肾病的诊断与治疗[J/OL]. 中华肾病研究电子杂志, 2013, 02(01): 28-33.

Jing-di SUN, Wen-ge LI, Jiu-yang ZHAO. The diagnosis and treatment of the IgG4-related kidney disease[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2013, 02(01): 28-33.

IgG4相关肾病肾损害的主要特征是肾小管间质肾炎,诊断依据主要包括:临床表现、血清学、影像学、组织病理学等指标。该病应用糖皮质激素治疗多数有效,越早治疗疗效越好,因此早期诊断非常重要。发病机制目前尚不完全清楚,可能主要与过敏或自身免疫功能紊乱有关。笔者就目前美国肾脏病学会及日本肾脏病学会的诊断标准进行探讨,以进一步加深对此病的认识。

IgG4-related kidney disease is characterized by tubulointerstitial nephritis. Its diagnoses rely on a combination of clinical, serological, radiographic, and histopathologic features.Response to corticosteroids therapy is favorable. The sooner, the better therapy. So early diagnosis is very important. However, the pathogenesis is still unclear,although related to allergy or autoimmune function disorders. Therefor, here we try to discuss about the diagnostic criteria of both American Society of Nephrology and Japanese Society of Nephrology,in order to deepen the understanding of this disease.

图1 IgG4相关肾病患者肾脏特异的影像学改变图像 注:a图为肾实质多发低密度灶;b图为弥漫性肾脏肿大;c图为单发肾脏肿瘤;d图为弥漫性肾盂壁肥厚(内腔光滑)
图2 IgG4肾病患者特异性肾脏病理组织改变图像 注:a图为片状淋巴细胞、浆细胞浸润及纤维化改变,病变部位与非病变部位界线清楚(PAM染色×40);b为特征性纤维化改变,即鸟眼征(PAM染色×100);c图、d图为明显的IgG4阳性浆细胞浸润(C:CD100染色×400,D:IgG4染色×400)
表1 美国肾脏病学会IgG4- TIN的推荐诊断标准[9]
表2 日本肾脏病学会IgG4-RKD诊断标准[10-11]
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