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

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. Key words: Pancreatitis; Autoimmune diseases; IgG4 cholangitis; Hormone therapy