Clinical characteristics of 27 patients with type 1 autoimmune pancreatitis

Objective To analyze the clinical characteristics of type 1 autoimmune pancreatitis (AIP) in patients. Methods The clinical data of 27 patients with type 1 AIP treated at Fujian Provincial Hospital from January 2012 to October 2018 were retrospectively analyzed. Results There are 25 males (92.6%) and 2 females (7.4%) (ratio 12.5∶1). The age of disease onset was (59.5±14.3) years. The most common presenting symptoms were jaundice and abdominal pain (both 59.3%). The most common complication was IgG4-related sclerosing cholangitis (63.0%). Magnetic Resonance Cholangiopancreatograhpy (MRCP) conducted on 26 patients showed the diffuse type 1 AIP was most common (53.8%), only 2 patients (7.7%) presented with a main pancreatic duct stricture. The pancreatic segment of bile duct narrowing was very common (84.6%), and most patients presented as tapered narrowings (65.4%). Standard glucocorticoid therapy was given to these 27 patients who responded well with clinical and laboratory remissions. Two patients were given maintenance glucocorticoid therapy for a high level of serum IgG4. Conclusions Type 1 AIP can present as a local manifestation of IgG4-related disease. The most common complication is IgG4-related sclerosing cholangitis. Glucocorticoid therapy was effective but some patients required maintenance therapy. Key words: Pancreatitis; Immunoglobulin G; Cholangiopancreatography, magnetic resonance; Autoimmune pancreatitis; Treatment outcome