Clinical Analysis Of 14 Cases Of IgG4-related Billiary And Pancreatic Diseases

Objective To analyze the clinical features of patients with IgG4-related biliary and pancreatic diseases,and to improve the understanding of these diseases.Methods Fourteen cases of patients with IgG4-related biliary and pancreatic diseases in Fujian Provincial Hospital from May 2012 to July 2015 were analyzed retrospectively, which included clinical manifestations, serological examination,imaging examination, pathology and treatment.Results The ratio of male to female was 13:1, with a mean age of(63.9± 10.5)years(range: 45-82 years).Seven cases were presented with jaundice, 6 cases with abdominal pain, 3 cases with physical examination found, 2 case with fatigue.One cases with primary biliary cirrhosis, 1 cases with immune vasculitis and 1cases with IgG4-related interstitial lung disease. There were 12 patients with high serum IgG4 which was twice as normal level, and 2 patients did not take blood test of IgG4. Five patients presented with elevated serum gammaglobulin. Serum Ig G levels were elevated in 8 cases, and 3 cases did not take serum Ig G. The CA199 of10 patients were elevated. Five cases of serum amylase levels were high,while 4cases did not take serum amylase. Five cases of anti nuclear antibody were positive,1 cases of anti extractable antigen antibody was positive,and 3 cases did not take their antibodies. CT examinations showed 5 cases with diffuse swelling of the pancreas, 5 cases had segmental swelling of the pancreas,1 case with bile duct dilatation only, and 1 case with bile duct thickening. MRCP examinations showed5 cases with pancreatic diffuse swelling, including pancreatic duct stricture in 3cases, and focal swelling in 5 cases,including pancreatic duct stricture in 2cases,and bile duct stricture in 8 cases. There were 8 cases confirming with IgG4-related autoimmune pancreatitis, 2 cases with IgG4-related sclerosing cholangitis, and 4 cases with IgG4-related autoimmune pancreatitis and IgG4-related sclerosing cholangitis. Eight patients received glucocorticoid treatment,3 patients underwent surgery and the surgical pathology indicated IgG4 positive plasma cell.Conclusions IgG4-related autoimmune pancreatitis and IgG4-related sclerosing cholangitis occurs mainly in older men, and jaundice and abdominal pain were the major clinical presentation. The diagnosis should combine clinical manifestations,other organ involvement, serological examination, imaging examination, and pathology. Glucocorticoid is an effective treatment.