摘要
目的：总结IgG4相关性疾病伴肾脏损害的临床特点。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,并复习相关文献。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Ig G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。
Objective： To summarize the clinical features of urinary system lesions in IgG4-related disease（ IgG4-RD）and review of literatures. Methods： Clinical manifestation,pathologic findings,laboratory profiles,treatments and prognosis of four IgG4-RD patients with urinary system involvement were analyzed retrospectively. Results： Four patients were males,51 ~ 69 years old. Urinary system lesion included hematuria,proteinuria,renal dysfunction and obstructive nephropathy. All the patients presented multiple organ involvement simultaneously,hyperglobulinemia,elevated serum Ig G and IgG4. CT image showed hypodense shadow of renal cortex,diffuse swelling of kidney,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 improvement of renal function and renal symptoms. Conclusions：Clinical manifestation of urinary lesions of IgG4-RD are varied,and can be well ameliorated with glucocorticoids.
出处
《内科急危重症杂志》
2015年第6期419-422,425,共5页
Journal of Critical Care In Internal Medicine
基金
国家自然科学基金项目(No:81100481
81500436)
