目的: 了解免疫球蛋白G4相关疾病(immunoglobulin G4-related disease,IgG4RD)患者的起病形式和就诊行为现况。方法: 采用流行病学研究方法,对在北京大学人民医院就诊的112例IgG4RD患者进行调查,其中资料完整者100例,内容包括患者的性别、年龄、身高、体重、病史、就诊过程及患病对工作的影响等。结果: 本组IgG4RD患者平均发病年龄为(51.51±12.9)岁,男女患病比为1 :0.75,女性发病年龄显著早于男性(P<0.001)。IgG4RD患者常见受累器官比例依次是颌下腺69%、泪腺59%、胰腺28%、腮腺28%,女性患者泪腺受累较多(P<0.05)。62%的患者合并有过敏性疾病。IgG4RD患者首诊科室主要为普通外科(9/100)、口腔及颌面外科(17/100)、风湿免疫科(16/100),确诊科室集中在风湿免疫科(67/100)、口腔颌面外科(16/100)和消化内科(7/100)。2010年后发病的患者平均发病到确诊月数较2010年前显著降低(P<0.01)。未明确诊断前接受过手术的患者有43例,首诊于外科的患者更易接受手术(P<0.01)。18%的患者因患病影响了工作。结论: IgG4RD女性患者并不少见,且发病年龄多早于男性,唾液腺、泪腺为最常见受累器官,且多为首发。过敏性疾病为IgG4RD常见的临床表现。临床漏诊、误诊普遍,近半数患者在诊断前接受了不必要的手术治疗,近年来延误诊断的情况略有改善,但仍应引起临床医师更多重视。

Objective: To comprehend clinical features and patient’s physician visit patterns at onset of immunoglobulin G4 related disease (IgG4RD). Methods: In the study, 100 patients with IgG4RD who received treatments in the Department of Rheumatology and Immunology of Peking University People’s Hospital from Apr. 1st, 2017 to Apr. 1st, 2018 were investigated, including gender, age, height, body weight, age of onset, physician visit history, primary history and how did the disease affected their life, etc. Results: In this 100 IgG4RD cohort (57 males and 43 females), the male/female ratio was 1 :0.75, the mean age of onset was (51.51±12.9) years, and the median duration was 49 months (ranging from 4 to 231 months). The onset age of males was significantly older than that of females (P<0.01). The manifestations showed that up to 69% patients had submaxillay glands lesion, 59% patients had lacrimal glands lesion, 28% patients had pancreas involvement and 28% patients had parotid glands involvement. The females had more lacrimal glands involvement (P<0.05). 62% patients were complicated with anaphylactic disease. The primary physician visit departments concentrated upon general surgery department (19/100), oral and maxillofacial surgery department (17/100), rheumatology and immunology department (16/100), ophthalmology department (15/100) and gastroenterology department (10/100). The departments where the confirmed diagnose was made concentrated upon rheumatology department (67/100),oral and maxillofacial surgery department (16/100) and gastroenterology department (7/100). The mean diagnosis duration after 2010 was (16.96±2.163) months, significantly shorter than that before 2010, which was (113.3±11.01) months. Before the definite diagnose was made, 43% patients underwent surgeries and 12% patients had more than one time surgeries. The patients whose first-visit department was a surgery department were more likely to undergo surgeries (P<0.01). 18% patients (18/100) stated that the disease had affected their work. Conclusion: In this cohort of the IgG4RD patients, female is common and has earlier onset age than male. The major manifestations of IgG4RD are salivary glands, lacrimal glands and pancreas involvement. The common chief complains are salivary glands and lacrimal glands enlargement. Accompanied by anaphylactic disease is a marked manifestation of this disease. Delayed diagnoses are not rare, though this situation has been improved since 2010, and more attention still should be paid to the disease.