目的: 了解临床表现复杂且容易被误诊和漏诊的IgG4相关性疾病(IgG4-related disease，IgG4-RD)患者的就诊情况及临床特征，以提高相关科室医师对IgG4-RD的认识。方法: 选择2012年1月1日至2022年12月31日于北京大学第三医院住院且出院诊断为IgG4-RD患者的病例资料进行回顾性分析，总结患者就诊情况、临床表现、实验室检查、诊断及治疗情况。结果: 共纳入出院诊断为IgG4-RD的病例116例，男女比例为2.52∶ 1，平均年龄(61.83±10.80)岁。首诊科室以消化科、普外科、眼科为主，诊断科室主要为消化科、风湿免疫科、呼吸科，21例(18.10%)患者经过3个或者3个以上科室诊断后才最终确诊。从出现临床症状到首次就诊中位时间为2(1，7)个月，诊断中位时间为1(1，12)个月。24例(20.69%)患者在最终诊断前进行了受累部位的手术切除。依据IgG4-RD的分类标准，确定诊断68例(58.62%)，可能诊断8例(6.90%)，可疑诊断40例(34. 48%)。68例确定诊断的患者中，常见受累器官依次为颌下腺、胰腺、胆道、腮腺，血清IgG4水平的中位数为6.16(3.61，12.30) g/L，57例(83.82%)患者使用了糖皮质激素，14例(20.59%)患者使用了免疫抑制剂，使用免疫制剂者主要为风湿免疫科诊治患者(78.57%)。结论: IgG4-RD好发于老年男性，颌下腺、胰腺、胆道、腮腺易受累，首诊科室分布广泛，通过获取病理组织标本而确定诊断的比例较低，治疗上主要以激素为主，免疫抑制剂的使用率不高。

Objective: To understand the medical treatment and clinical characteristics of patients with IgG4-related disease (IgG4-RD) with complex clinical manifestations and easy to be misdiagnosed and missed, and to improve the recognition of this disease among doctors from relevant medical departments. Methods: A retrospective analysis was conducted on the medical records of patients diagnosed with IgG4-RD who were hospitalized and discharged from Peking University Third Hospital from January 1, 2012 to December 31, 2022. The patient' s medical visit status, clinical manifestations, laboratory examinations, diagnosis, and treatment information were summarized. Results: A total of 116 patients diagnosed with IgG4-RD were included in this study, with a male to female ratio of 2. 52∶ 1 and an average age of (61.83±10.80) years. The departments for initial visits were gastroenterology, general surgery, and ophthalmology. While the departments responsible for definitive diagnosis were gastroenterology, rheumatology and immunology, and respiratory medicine. Twenty-one patients (18. 10%) required consultation and treatment from three or more departments before receiving a definitive diagnosis. The median time from symptom onset to the initial clinic visit was 2 (1, 7) months, and the median time from symptom onset to diagnosis was 1 (1, 12) month. Twenty-four patients (20.69%) underwent surgical resection of the affected sites before diagnosis. According to the classification criteria of IgG4-RD, sixty-eight (58.62%) cases were diagnosed definitively, eight (6.9%) cases were likely to be diagnosed, and 40 (34.48%) cases were suspected to be diagnosed. In the 68 definitively diagnosed patients, the most commonly affected organs were submandibular gland, the pancreas, biliary tract, parotid in sequence. The median serum IgG4 (IgG4, immunoglobulin G4) level was 6.16 (3. 61, 12. 30) g/L. Fifty-seven patients (83.82%) were treated with glucocorticoids, and 14 patients (20.59%) were treated with immunosuppressants. The use of immunosuppressants was mainly in the rheumatology and immunology department (78. 57%). Conclusion: IgG4-RD is more common in elderly males, with submandibular gland, the pancreas, biliary tract, and parotid being most commonly affected. The distribution of initial visit departments in patients is wide. The proportion of definitive diagnosis based on pathology is relatively low. In terms of treatment, the main approach is steroid treatment, while the use of immunosuppres-sants is not widespread.