隆沐阳 刘云 刘岩

    【摘要】 IgG4相关性肾病(IgG4-RKD)是近年才被认识的一种免疫相关性疾病，该病常累及肾小管，也可累及肾小球，对肾上腺皮质激素(激素)治疗敏感，确切的发病原因尚不十分清楚。该文报告1例以皮肤瘙痒、突眼9年余及排泡沫尿3个月为主诉的中年女性患者，其有甲状腺结节，血清IgG4升高(达到19 700 mg/L)，肾脏活组织检查呈现典型病理征(免疫组织化学染色可见间质较多浆细胞浸润，IgG4+浆细胞>10个/高倍视野)，诊断考虑IgG4-RKD(膜性肾病为主)。予激素治疗后患者临床症状明显缓解。该例的诊治过程提示，在临床诊治过程中遇到反复过敏、腎功能异常及相关器官组织纤维化的患者时应注意排除IgG4-RKD，重视相关鉴别诊断，对于过敏性体质的患者有必要常规进行过敏原的筛查，尽早完善IgE、IgG4、影像学和组织病理学等检查，关注及评估患者是否合并多系统损伤，以早期干预及治疗。

    【关键词】 IgG4相关性疾病;膜性肾病;过敏;诊断;治疗

    【Abstract】 IgG4-related kidney disease (IgG4-RKD) is a newly recognized immune-related disease， which is involved with renal tubules and glomerulus. IgG4-RKD is sensitive to glucocorticoid (hormone) therapy. The exact pathogenesis of IgG4-RKD is still unclear. This article reported a middle-aged female patient who presented with skin pruritus and exophthalmos for over nine years and foamy urine for three months as chief complaints. This patient was diagnosed with thyroid nodules. The serum level of IgG4 was elevated up to 19 700 mg/L. Renal biopsy revealed typical pathological features (immunohistochemical staining detected dense plasmacytic infiltration in the renal stroma with infiltrating IgG4+ plasma cells > 10/high-power field). The diagnosis of IgG4-RKD (mainly the membranous nephropathy) was considered. After hormone treatment ......