IgG4相关性腹部疾病31例临床分析

Clinical analysis of 31 cases of IgG4-related abdominal disease

目的分析总结IgG4相关性腹部疾病临床特征和诊治经过,以提高对本病诊治水平。方法回顾性分析同济医院2010年1月至2020年9月31例IgG4相关性腹部疾病患者的临床资料。结果31例IgG4相关性腹部疾病患者中,男性27例(87.10%),女性4例(12.90%),年龄(67.26±6.16)岁。临床表现为腹痛不适21例(67.74%),黄疸及肝功能异常4例(12.90%),肾功能异常4例(12.90%),腰痛1例(3.23%),腹膜后肿块1例(3.23%)。疾病诊断中,IgG4相关自身免疫性胰腺炎21例(67.74%),IgG4相关硬化性胆管炎4例(12.90%),IgG4相关肾病4例(12.90%),IgG4相关腹膜后纤维化2例(6.45%)。25例患者接受糖皮质激素治疗,3例停药后复发,再次使用醋酸泼尼松或联合免疫抑制剂治疗,症状好转,复查IgG4水平下降。6例患者接受手术治疗,无1例死亡。结论IgG4相关性腹部疾病少见,临床认识普遍不足。腹部病变以IgG4相关自身免疫性胰腺炎为主,具有特征性影像学表现。糖皮质激素疗效较好,但停用后存在复发风险。

Objective To analyze and summarize the clinical characteristics,diagnosis and treatment of IgG4-related abdominal disease,and improve the level of diagnosis and treatment of IgG4-related abdominal disease.Methods The clinical data of 31 patients with IgG4-related abdominal disease in Tongji Hospital from January 2010 to September 2020 were analyzed retrospectively.Results Among the 31 patients with IgG4-related abdominal disease,there were 27 males(87.10%)and 4 females(12.90%),aged(67.26±6.16)years.The clinical manifestations were abdominal pain and discomfort(21 cases,67.74%),jaundice and abnormal liver function(4 cases,12.90%),abnormal renal function(4 cases,12.9%),low back pain(1 case,3.23%),and retroperitoneal mass(1 case,3.23%).In the diagnosis,there were 21 cases of IgG4 related autoimmune pancreatitis(67.74%),4 cases of IgG4 related sclerosing cholangitis(12.90%),4 cases of IgG4 related nephropathy(12.90%),and 2 cases of IgG4 related retroperitoneal fibrosis(6.45%).A total of 25 patients received glucocorticoid treatment,among them there were 3 patients relapsed after drug withdrawal and they were treated with prednisone acetate or combined with immunosuppressant again and the symptoms improved and the level of IgG4 decreased in reexamination.There were 6 patients received surgical treatment,and none died.Conclusion IgG4-related abdominal disease is rare,and the clinical understanding is generally insufficient.Abdominal lesions were mainly IgG4 related autoimmune pancreatitis with characteristic imaging findings.Glucocorticoid is effective,but there is a risk of recurrence after discontinuation.