雒真龙,潘昊,裴广畅,代维,李月强,汪志祥,韩敏,何晓峰,吕永曼,高红宇.IgG4相关性疾病伴肾脏损害病例报告并文献复习[J].内科急危重症杂志,2015,21(6):
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| DOI： |
| 中文关键词: IgG4相关性疾病 肾功能异常 泌尿系统 糖皮质激素 |
| 英文关键词:IgG4-RD renal dysfunction urinary system glucocorticoids |
| 基金项目:基金号81100481；81500436 |
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| 中文摘要: |
| 目的 总结IgG4相关性疾病伴肾脏损害疾病的临床特点并复习相关文献。方法 回顾性分析4例IgG4相关性疾病伴肾脏损害患者的临床表现、病理特点、实验室检查、治疗及预后情况。结果4例患者均为男性，年龄51-69岁，泌尿系统损害包括不同程度的血尿、蛋白尿、肾功能异常、梗阻性肾病。4例患者均同时存在泌尿系统外的多器官受累。所有患者均存在高球蛋白血症，血清IgG及IgG4亚型显著升高。影像学表现可分为4类：肾皮质低密度影、肾脏弥漫增大、肾盂和(或)输尿管积水、腹膜后纤维化。肾脏病理显示肾间质大量淋巴细胞、IgG4阳性的浆细胞浸润伴纤维化表现。患者对糖皮质激素治疗反应良好，临床症状及肾功能均明显改善。结论 累及泌尿系统的IgG4相关性疾病临床表现复杂多样，糖皮质激素治疗可较快缓解病情。 |
| 英文摘要: |
| Objective To summarize the clinical features and review the literature of urinary system lesions in IgG4-related disease (IgG4-RD)．Methods Clinical manifestation, pathologic findings, laboratory profiles, treatment and prognosis of 4 IgG4-RD patients with urinary system involvement were analyzed．Results All four patients were males with aged 51-69 years old．Urinary system lesion included hematuria, proteinuria, renal dysfunction, obstructive nephropathy. All the patients presented multiple organ involvement simultaneously, hyperglobulinemia, elevated serum IgG and IgG4. CT image often showed hypodense shadow with renal cortex, renal swelling, hydronephrosis, and retroperitoneal fibrosis．Renal pathology revealed predominantly inflammatory cells infiltration comprising lymphocytes and IgG4-positive plasma cells with fibrosis. Patients responded well to glucocorticoids with the renal function and symptoms improved．Conclusions Urinary lesions of IgG4-RD are varied form in clinical manifestation, and can be well ameliorated with glucocorticoids. |
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