Abstract
AIMS: To provide comprehensive data on the diagnosis and treatment of autoimmune pancreatitis (AIP) patients in China. DESIGN: A systematic review. METHODS: All clinical studies concerning AIP from China published between January 2006 and June 2014 were retrospectively reviewed and analyzed. RESULTS: A total of 26 original articles involving 706 AIP patients were included with an estimated proportion of type 2 AIP as 4.7%. In the 706 AIP patients, the range of mean/median age was 48.6-67.0 years old and the male to female ratio was 4.47:1. The common presentations included obstructive jaundice (pooled rate: 63.4%, 95%CI: 55.4%-71.0%) and abdominal symptoms (pooled rate: 62.3%, 95%CI: 52.4%-71.7%). Biliary involvement was the most common extrapancreatic manifestations, especially the lower part of the common bile duct (pooled rate: 62.3%, 95%CI: 49.9%-73.9%). According to the imaging examinations, 53.8% and 41.6% patients were classified into focal-type and diffuse-type, respectively. Notably, upstream pancreatic duct dilatation was found in parts of patients (pooled rate: 13.8%, 95%CI: 6.6%-23.1%). The levels of serum IgG4 were elevated in most patients (pooled rate: 86.0%, 95%CI: 74.2%-94.6%). Nearly three tenths AIP patients received surgery (pooled rate: 29.7%, 95%CI: 18.1%-42.8%) due to mimicked malignancy. Steroid treatment was given to 78.4% patients (95%CI: 65.3%-89.1%) with a pooled remission rate of 96.2% (95%CI: 94.0%-97.9%). The pooled relapse rate was 13.8% (95%CI: 7.2%-22.0%) with the mean follow-up time ranging from 12 to 45 months. CONCLUSION: Type 1 is the predominant type of Chinese AIP patients and the clinical features, diagnostic modalities and therapeutic regimen were similar with those in other countries. Knowledge of AIP should be more widespread to avoid unnecessary surgery.