IgG4相关胆胰疾病九例临床分析

Clinical analysis of 9 cases of IgG4-associated biliary and pancreatic diseases

目的 分析IgG4相关胆胰疾病患者的临床特征,提高对此类疾病的认识.方法 回顾性分析9例IgG4相关胰腺炎及胆管炎患者的临床表现,血清学、影像学、病理学检查结果,治疗及预后情况.结果 9例患者中男性8例,女性1例,年龄40～78岁,平均61岁.4例临床表现为黄疸,2例为黄疸及腹痛,2例为腹痛,1例表现为糖尿病.6例血清IgG4为3.46 ～21.3 g/L,平均9.743 g/L,均高于正常值;3例未查血清IgG4.9例患者血清19项自身抗体均阴性,4例CA19-9升高.5例CT表现为胰腺低密度占位,其中2例合并胆管扩张;1例CT表现为胰腺肿胀伴胆管扩张;2例单纯胆管扩张;1例CT无阳性发现.4例行EUS-FNA检查,EUS下主要表现为低回声病变,胰周均未见肿大淋巴结.FNA病理检查结果提示2例为IgG4相关慢性炎症,2例为IgG4阴性的慢性炎症.7例确诊为IgG4相关胰腺炎,2例为IgG4相关胆管炎.6例行激素治疗,强的松8～ 40 mg/d;3例手术治疗,术后病理检查均见IgG4阳性浆细胞.出院后随访复查血清IgG,4均恢复正常,临床症状也明显改善,胰腺占位不同程度缩小.结论 IgG4相关胰腺炎及胆管炎是以IgG4阳性浆细胞浸润为主要表现的自身免疫性疾病,诊断需结合影像学、血清学、病理学等检查,糖皮质激素治疗有效.

Objective To analyze the clinical features of patients with IgG4-associated biliary and pancreatic diseases,and to improve the understanding of these diseases.Methods Nine cases of patients with IgG4-associated pancreatitis and cholangitis were analyzed retrospectively,which included clinical manifestations,serological examination,imaging test,pathology,treatment and prognosis.Results Of the 9 patients,8 were male,1 was female,the average age was 61 years old.Four cases were presented with jaundice,2 cases with jaundice and abdominal pain,2 cases with abdominal pain,and 1 case with diabetes.Serum IgG4 levels were 3.46-21.3 g/L （mean 9.743 g/L） in 6 cases,and which were higher than normal value,3 patients did not undergo blood test of IgG4.Nineteen auto-antibodies （including ANCA） were all negative in 9 cases.CA19-9 was increased in 4 cases.CT manifestations showed 5 cases with low density pancreas mass （2 cases with biliary tract dilatation）;1 case had pancreas swelling with dilatation of bile duct,and 2 cases with bile duct dilatation only,and CT findings in 1 case were negative.Four patients underwent EUS-FNA,and EUS features included hypoechoic lesions without peripancreatic lymph nodes.FNA results indicated 2 cases with IgG4 related chronic inflammation,2 cases with chronic inflammation with negative IgG4.Seven cases were confirmed to have IgG4 related pancreatitis,and 2 cases with IgG4 related cholangitis.Six patients received glucocorticoid treatment,and the dose ranged from 8-40 mg;3 patients underwent surgery and the surgical pathology indicated IgG4 positive plasma cell.Follow-up showed the serum IgG4 returned to normal,clinical symptoms improved remarkably,and pancreatic mass decreased.Conclusions IgG4 positive plasma cell infiltration is the main feature of IgG4-associated cholangitis and pancreatitis.The diagnosis should combine image,serology with pathology.Glucocorticoid is an effective treatment.