IgG4相关疾病的临床病理分析

Clinical and pathological characteristics of IgG4 related disease

目的:总结IgG4相关疾病(IgG4-RD)器官受累的临床病理特点。方法:回顾性分析2012年至2017年南京总医院收治的IgG4-RD患者的临床表现、病理、治疗及预后特点。结果:共入组32例患者,男性26例、女性6例,平均发病年龄57.3±13.8岁。20例伴脏器肿大,最常见受累脏器为肾脏(17/32)、胰腺(7/32)、颌下腺(8/32)等。100%(29/29)血IgG4升高,25%(8/32)嗜酸性粒细胞比例升高,62.5%(21/32)球蛋白升高,72.7%(16/22) IgG升高,77.3%(17/22)血IgE升高,36.4%(8/22)有低补体血症。与无肾脏受累组相比,肾脏受累组贫血更重,IgG4-RD应答指数更高;与补体正常组相比,低补体组IgG4水平更高。12例患者行肾脏活检,10例表现为肾小管间质性肾炎,2例为合并肾小球膜性病变。肾脏受累的患者,15例接受糖皮质激素或免疫抑制剂治疗,尿检或肾功能均改善。结论:IgG4-RD为多系统炎症性疾病,临床表现多样,肾脏受累以肾小管间质性肾炎为主,多数患者糖皮质激素治疗反应良好。

Objective：To summarize the clinical and pathological features of IgG4- related diseases （IgG4-RD）. Methodology ：The clinical manifestations ,pathological features ,treatment and prognosis of IgG4-RD patients admitted to Jinling hospital from 2012 to 2017 were analyzed retrospectively. Results： There were 26 male and 6 female with an average age of （57.3± 13.8） years old.20/32 cases had organs swelling,and the most common affected organs were kidney （ 17/32）, pancreas （ 7/32 ）, submandibular gland （ 8/31 ）, etc. Increase of serum IgG4 was in 29/29, peripheral blood eosinophils in 8/32, globulin in 21/32, IgG in 16/22, IgE in 17/22,and low serum C3 and C4 levels in 8/22.Renal damage was characterized by abnormal urine tests ,enlarged kidneys and renal dysfunction.Compared with the no renal involvement group, there was more severe anemia and higher responder in renal involvement group. Compared with the normal complement group, the IgG4 level was higher in the low complement group, 12 patients underwent renal biopsy, the histological diagnosis was IgG4-related tubulointerstitial nephritis （TIN） in 10 cases, membranous glomerulonephropathy in 2 cases. 15 cases with renal involvement treated with glucocorticoid or immunosuppressive agents all showed improvement in urine test or renal function. Conclusion： IgG4-RD is a multi-systemic inflammatory disease with multiple clinical manifestations,and renal involvement is mainly characterized by TIN.Most patients respond well to glucocorticoid therapy.