Clinical analysis of 9 cases of IgG4-associated biliary and pancreatic diseases / 中华胰腺病杂志

ABSTRACT

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.