免疫球蛋白G4相关疾病150例治疗前后实验室检测数据的比较

Comparison of laboratory tests of 150 patients with immunoglobulin G4-related diseases

目的分析免疫球蛋白（Ig）G4相关疾病（IgG4-RD）的临床特征和治疗前后实验室数据，提高临床对此疾病的认识。方法总结北京协和医院IgG4．RD前瞻性队列研究中自2010年1月至2014年10月间纳入的150例患者资料，治疗前全面评估，治疗后在第1、3、6-10个月时进行3次随访。治疗前后符合正态分布的数据采用配对t检验，非正态分布的数据采用非参数检验的Wilcoxon秩和检验。结果本组患者男：女比例为2．33：1，平均年龄（53±12）岁。87例（58．0％）患者曾有过敏史。临床表现为多器官受累，前3位最常见的表现为米库利兹病（MD）、淋巴结病、胰腺炎，均接受糖皮质激素或糖皮质激素联合免疫抑制剂治疗。MD组患者IgG4水平显著高于非MD组，补体C3、C4水平显著低于非MD组。治疗前分别有70.3％和88．2％的患者IgG和I球升高，治疗后显著下降。治疗前95．3％的患者血IgG4水平升高为8110．0（3242．5，18325．0）mg／L；治疗后第1、2、3次随访临测IgG4水平分别为5195（1962．5，11300）mg／L、3170（897，7410）mg／L、3010（1095，6670）mg／L，均较治疗前有显著下降（U=-7．514，-9．028，-9．031，均P〈0．01）；治疗后32．6％的患者IgG4正常。IgG4与IgE的水平无相关性，治疗后的变化趋势非平行性。结论IgG4-RD实验室检查中以血清IgG4升高最突出，治疗后IgG4水平明娩下降，但多数患者不能降至正常；该疾病与过敏性疾病有一定相关性。治疗后IgE与IgG4变化趋势并不一致。

Objective To explore the clinical and laboratory characteristics of immunoglobulin G4- related diseases （IgG4-RD）. Methods A prospective cohort study on IgG4-RD patients was carried out in Peking Union Medical College Hospital during January 2010 to October 2014, a total of 150 patients were recl＇uited. The laboratory features before treatment and 3 times follow up which were at the first, 3rd, 6-10th months after treatment were summarized and analyzed. Normal distributed data before and after treatment was statistically compared by paired t-test while non-normal distributed data was tested by Wilcoxon rank sum test. Results The male-to-female ratio and age was 2.33：1 and （53±12） years old respectively. Allergic histories were reported in 87 subjects （58.0%）. Multiple organ involvements were documented and the top three most common manifestations were Mikulicz＇s disease （MD）, lymph node diseases and autoimmune pancreatitis, the vast majority of cases accepted glucocorticosteroid with/without immune suppressive treatment, The level of IgG4 in the MD group was significantly higher than that in the non-MD group, while the level of complement C4 and C3 was significantly lower. Seventy point three percent and 88.2% of the patients had elevated IgG and IgE levels, which decreased significantly after treatment. The serum IgG4 levels were elevated in 95.3% cases with 8 110 （3 242.5, 18 325.0） mg/L before treatment. After treatment, the IgG4 levels decreaseed to 5 195（1 962.5, 11 300） rag/L, 3 170（897, 7410） mg/L, 3 010（1 095, 6 670） mg/L, at three followed-up time points respectively （U=-7.514, -9.028, -9.031, P〈0.01）, with 32.6% cases returned to normal level. IgG4 level had no correlation with IgE and the trend of their change was not parallel with treatment. Conclusion The elevated serum IgG4 level is the most prominent laboratory characteristics of IgG4-RD. It decreases significantly after treatment, but may not reduce to normal level in most patients. IgG4-RD and allergic disease has a certain correlation. The trend of IgE and IgG4 change is not parallel with treatment.