To analyze the clinical features, diagnosis and treatment in patients with immunoglobulin 4 (IgG4)-related diseases. Methods: The clinical data (including general situation, clinical manifestations, laboratory examination, imaging examination, pathological examination, treatment and follow-up) in 21 patients, who were diagnosed with IgG4-related diseases in Second Xiangya Hospital of Central South University from June 2014 to February 2018, were retrospectively analyzed. Results: Among the 21 patients, including men 16 (76.2%) and women 5 (23.8%), the age was 37.00-78.00 (59.19±12.93) years old. Multi-organ involvement was discovered in 10 patients (47.6%), among which the lung (42.9%), pancreas (38.1%), kidney (33.3%), bile duct (19.0%) and liver (14.3%) were the main organs involved. The serum IgG4 levels in 19 patients (90.5%) were increased [8.63 (1.13-36.50) g/L]. The condition of 16 patients (84.2%) was improved after glucocorticoid therapy. Conclusion: IgG4-related diseases are mostly found in middle-aged and elderly men. Multiple organs or tissues of the body can be involved. Affected organs are mostly seen in lung, pancreas, kidney and bile duct, with diverse clinical manifestations. Glucocorticoid and immunosuppressive therapy are effective.
目的：总结IgG4相关性疾病患者的临床特点、诊断及治疗。方法：对中南大学湘雅二医院2014年6月至2018年2月诊断为IgG4相关性疾病的21名患者的临床资料进行回顾性分析，这些资料包括患者的一般情况和临床表现，实验室检查、影像学检查、病理学检查结果，治疗及随访。结果：21例患者中，男16例(76.2%)，女5例(23.8%)；年龄为37.00~78.00(59.19±12.93)岁；10例(47.6%)出现多器官受累，其中肺(42.9%)、胰腺(38.1%)、肾(33.3%)、胆管(19.0%)、肝(14.3%)等为主要受累器官；19例(90.5%)患者血清IgG4[8.63(1.13~36.50) g/L]升高；16例(84.2%)患者在糖皮质激素治疗后病情改善。结论：IgG4相关性疾病多见于中老年男性，身体多个器官或组织均可受累，受累器官以肺、胰腺、肾及胆管等多见，临床表现多样，糖皮质激素及免疫抑制剂治疗有效。.