Feng Lu, Zhai Jia Yu, Zhao Jin Xia
Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing 100191, China.
Department of Rheumatology and Immunology, Liaoning Health Industry Group Bengang General Hospital, Benxi 117000, Liaoning, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2023 Dec 18;55(6):1028-1032. doi: 10.19723/j.issn.1671-167X.2023.06.011.
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.
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.
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%).
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.
了解IgG4相关性疾病（IgG4-RD）患者临床表现复杂、易误诊漏诊的医疗救治及临床特点，提高相关临床科室医生对该病的认识。
回顾性分析2012年1月1日至2022年12月31日在北京大学第三医院住院并出院的确诊为IgG4-RD患者的病历资料。总结患者就诊情况、临床表现、实验室检查、诊断及治疗信息。
本研究共纳入116例确诊为IgG4-RD的患者，男女比例为2.52∶1，平均年龄（61.83±10.80）岁。初诊科室为消化内科、普通外科和眼科。而负责明确诊断的科室为消化内科、风湿免疫科和呼吸内科。21例（18.10%）患者在明确诊断前需要三个或更多科室会诊及治疗。从症状出现到首次就诊的中位时间为2（1，7）个月，从症状出现到确诊的中位时间为1（1，12）个月。24例（20.69%）患者在诊断前行了受累部位手术切除。根据IgG4-RD分类标准，确诊68例（58.62%），可能诊断8例（6.9%），疑似诊断40例（34.48%）。在68例确诊患者中，最常受累器官依次为颌下腺、胰腺、胆道、腮腺。血清IgG4（免疫球蛋白G4）水平中位数为6.16（3.61，12.30）g/L。57例（83.82%）患者接受糖皮质激素治疗，14例（20.59%）患者接受免疫抑制剂治疗。免疫抑制剂的使用主要在风湿免疫科（78.57%）。
IgG4-RD在老年男性中较为常见，最常受累的是颌下腺、胰腺、胆道和腮腺。患者初诊科室分布广泛。基于病理明确诊断的比例相对较低。在治疗方面，主要治疗方法是激素治疗，而免疫抑制剂的使用并不广泛。
