Abstract：
OBJECTIVE: The aim of this study was to observe the demographic and clinical characteristics of immunoglobulin (Ig) G4-related disease (IgG4-RD). We aimed to compare different treatment methods and to identify the risk factors for non-response and relapse after treatment.
METHODS: We performed a retrospective study of 201 IgG4-RD patients initially diagnosed and treated at the First Affiliated Hospital of China Medical University from January 2016 to December 2020. Patients\' sex, age, clinical manifestations, baseline biochemical values, the number of organs involved, and the type of organ involvement were recorded. All patients received glucocorticoid (GC) monotherapy or GC + immunosuppressant combination therapy. The serum IgG4 concentration as well as the details of clinical response, relapse, and side effects were recorded at 1, 3, 6, and 12 months after treatment.
RESULTS: The incidence of IgG4-RD was primarily centered in the age group of 50-70 years old, and the proportion of affected male patients increased with age. The most common clinical symptom was swollen glands or eyes (42.79%). The rates of single- and double-organ involvement were 34.83% and 46.27%, respectively. The pancreas (45.77%) was the most frequently involved organ in cases of single-organ involvement, and the pancreas and biliary tract (45.12%) was the most common organ combination in cases of double-organ involvement. Correlation analysis showed that the number of organs involved was positively related to the serum IgG4 concentration (r = 0.161). The effective rate of GC monotherapy was 91.82%, the recurrence rate was 31.46%, and the incidence of adverse reactions was 36.77%. Meanwhile, the effective rate of GC + immunosuppressant combination therapy was 88.52%, the recurrence rate was 19.61%, and the adverse reaction rate was 41.00%. There were no statistically significant differences in response, recurrence, and adverse reactions. The overall response rate within 12 months was 90.64%. Age (< 50 years old) and aorta involvement were significantly associated with non-response. The overall recurrence rate within 12 months was 26.90%. Age (< 50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement were significantly associated with recurrence.
CONCLUSIONS: The clinical features vary among different age groups and according to gender. The number of organs involved in IgG4-RD is related to the serum IgG4 concentration. Age (< 50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement are risk factors for recurrence.
METHODS: We performed a retrospective study of 201 IgG4-RD patients initially diagnosed and treated at the First Affiliated Hospital of China Medical University from January 2016 to December 2020. Patients\' sex, age, clinical manifestations, baseline biochemical values, the number of organs involved, and the type of organ involvement were recorded. All patients received glucocorticoid (GC) monotherapy or GC + immunosuppressant combination therapy. The serum IgG4 concentration as well as the details of clinical response, relapse, and side effects were recorded at 1, 3, 6, and 12 months after treatment.
RESULTS: The incidence of IgG4-RD was primarily centered in the age group of 50-70 years old, and the proportion of affected male patients increased with age. The most common clinical symptom was swollen glands or eyes (42.79%). The rates of single- and double-organ involvement were 34.83% and 46.27%, respectively. The pancreas (45.77%) was the most frequently involved organ in cases of single-organ involvement, and the pancreas and biliary tract (45.12%) was the most common organ combination in cases of double-organ involvement. Correlation analysis showed that the number of organs involved was positively related to the serum IgG4 concentration (r = 0.161). The effective rate of GC monotherapy was 91.82%, the recurrence rate was 31.46%, and the incidence of adverse reactions was 36.77%. Meanwhile, the effective rate of GC + immunosuppressant combination therapy was 88.52%, the recurrence rate was 19.61%, and the adverse reaction rate was 41.00%. There were no statistically significant differences in response, recurrence, and adverse reactions. The overall response rate within 12 months was 90.64%. Age (< 50 years old) and aorta involvement were significantly associated with non-response. The overall recurrence rate within 12 months was 26.90%. Age (< 50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement were significantly associated with recurrence.
CONCLUSIONS: The clinical features vary among different age groups and according to gender. The number of organs involved in IgG4-RD is related to the serum IgG4 concentration. Age (< 50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement are risk factors for recurrence.
摘要：
目的：本研究的目的是观察免疫球蛋白（Ig）G4相关疾病（IgG4-RD）的人口统计学和临床特征。我们旨在比较不同的治疗方法，并确定治疗后无反应和复发的危险因素。
方法：我们对2016年1月至2020年12月在中国医科大学附属第一医院初次诊断和治疗的201例IgG4-RD患者进行了回顾性研究。病人性，年龄,临床表现，基线生化值，涉及的器官数量，并记录器官受累的类型。所有患者均接受糖皮质激素（GC）单药治疗或GC免疫抑制剂联合治疗。血清IgG4浓度以及临床反应的细节，复发,并在治疗后1、3、6和12个月记录副作用。
结果：IgG4-RD的发生率主要集中在50-70岁的年龄组，受影响的男性患者比例随年龄增长而增加。最常见的临床症状是肿胀的腺体或眼睛（42.79％）。单、双器官受累率分别为34.83%和46.27%,分别。在单器官受累的病例中,胰腺(45.77%)是最常受累的器官,胰腺和胆道（45.12％）是双器官受累病例中最常见的器官组合。相关性分析显示受累器官数量与血清IgG4浓度呈正相关(r=0.161)。GC单药治疗有效率为91.82%,复发率为31.46%,不良反应发生率为36.77%。同时,GC+免疫抑制剂联合治疗有效率为88.52%,复发率为19.61%,不良反应发生率为41.00%。反应没有统计学上的显著差异，复发,和不良反应。12个月内的总有效率为90.64%。年龄（<50岁）和主动脉受累与无反应显着相关。12个月内总复发率为26.90%。年龄（<50岁），低血清C4浓度，大量涉及的器官，淋巴结受累与复发显著相关。
结论：临床特征因不同年龄段和性别而异。IgG4-RD涉及的器官的数量与血清IgG4浓度有关。年龄（<50岁），低血清C4浓度，大量涉及的器官，淋巴结受累是复发的危险因素。
方法：我们对2016年1月至2020年12月在中国医科大学附属第一医院初次诊断和治疗的201例IgG4-RD患者进行了回顾性研究。病人性，年龄,临床表现，基线生化值，涉及的器官数量，并记录器官受累的类型。所有患者均接受糖皮质激素（GC）单药治疗或GC免疫抑制剂联合治疗。血清IgG4浓度以及临床反应的细节，复发,并在治疗后1、3、6和12个月记录副作用。
结果：IgG4-RD的发生率主要集中在50-70岁的年龄组，受影响的男性患者比例随年龄增长而增加。最常见的临床症状是肿胀的腺体或眼睛（42.79％）。单、双器官受累率分别为34.83%和46.27%,分别。在单器官受累的病例中,胰腺(45.77%)是最常受累的器官,胰腺和胆道（45.12％）是双器官受累病例中最常见的器官组合。相关性分析显示受累器官数量与血清IgG4浓度呈正相关(r=0.161)。GC单药治疗有效率为91.82%,复发率为31.46%,不良反应发生率为36.77%。同时,GC+免疫抑制剂联合治疗有效率为88.52%,复发率为19.61%,不良反应发生率为41.00%。反应没有统计学上的显著差异，复发,和不良反应。12个月内的总有效率为90.64%。年龄（<50岁）和主动脉受累与无反应显着相关。12个月内总复发率为26.90%。年龄（<50岁），低血清C4浓度，大量涉及的器官，淋巴结受累与复发显著相关。
结论：临床特征因不同年龄段和性别而异。IgG4-RD涉及的器官的数量与血清IgG4浓度有关。年龄（<50岁），低血清C4浓度，大量涉及的器官，淋巴结受累是复发的危险因素。
