IgG4相关性疾病泌尿系统损害分析

Analysis on urinary system lesions of IgG4-related disease

目的总结IgG4相关性疾病泌尿系统损害的临床特点，以其提高对该疾病的认识。方法回顾性分析出现泌尿系统损害的IgG4相关性疾病患者6例的临床表现、实验室检查、影像学资料、病理表现、治疗及预后情况。结果诊断为IgG4相关性疾病患者中6例存在泌尿系统损害，男女比例为4：2，中位年龄59岁（36—72岁），中位病程为10．5个月。除肾脏、输尿管受累外，所有患者均同时存在泌尿系统外的多器官受累。泌尿系统损害临床表现多样，包括肾功能异常、水肿和腹痛。所有患者均存在高球蛋白血症、血清IgG（中位值23.3g／L）及IgG4亚型（中位值4227．0mg／L）升高，肾小管源性蛋白尿；5例患者Scr明显升高（中位值237μmol／L）。影像学表现可分为4类：肾脏弥漫增大、CT多发低密度灶可伴不均匀强化灶、肾盂和（或）输尿管积水、肾脏萎缩。肾脏病理显示为弥漫纤维化伴肾间质大量淋巴细胞、浆细胞浸润的间质性肾炎表现，伴淋巴细胞、浆细胞IgG4免疫组化染色阳性。患者对中至大剂量糖皮质激素治疗反应良好，经治疗，临床症状改善，IgG、IgG4及Scr均明显降低。结论IgG4相关性疾病泌尿系统损害临床表现多样化，多同时合并其他器官受累；肾组织病理学以IgG4阳性的淋巴细胞和浆细胞浸润的间质性肾炎为其突出特点；糖皮质激素治疗有效。

Objective To explore the clinical features of renal and urinary lesions in IgG4- related disease （IgG4- RD）. Methods Clinical manifestation, laboratory profiles, iconography images, pathologic findings, treatment and prognosis of 6 IgG4- RD patients with renal and urinary system involvement from Peking Union Medical College Hospital during Aug 2010 to Dec 2011 were analyzed retrospectively. Results Six patients had renal and/or urinary lesions among IgG4- RD cases diagnosed in our hospital, including 4 males and 2 women, with median age of 59 years （36 to 72 years） and median disease course of 10.5 months. All the patients presented multiple organ involvement simultaneously. Urinary system lesion varied, including renal dysfunction, abdominal pain and edema. Hyperglobulinemia, elevated serum IgG （median 23.3 g/L） and IgG4 （median 4227.0 mg/L）, tubular proteinuria were found in all the 6 patients, and elevated Scr （median 237 μmol/L） in 5 cases. Kidney CT image often showed renal swelling, hydronephrosis, multiple low density focus with attenuation and kidney atrophy. Renal pathology revealed interstitial inflammatory cells infiltration comprising predominantly plasma cells and lymphocytes, with a high prevalence of IgG4- positive cells often admixed with fibrosis, which fit the features of tubulointerstitial nephritis. Patients with IgG4- RD nephropathy presented good response to glucocorticoids. After therapy, the symptoms were improved and serum IgG, IgG4 and Scr decreased. Conclusions Renal and urinary lesions of IgC,4-RD are heterogeneous in clinical manifestation, and are often complicated with various organ lesions. The feature of renal histopathology is tubulointerstitial nephritis infiltrated by plasma cells and lymphocytes with positive IgG4. Glueocorticoids treatment is effective for this disease.