Clinical Diagnosis and Treatment of Autoimmune Pancreatitis and IgG4-related Sclerosing Cholangitis / 胃肠病学

ABSTRACT

Background: Autoimmune pancreatitis (AIP) is a chronic pancreatitis associated with immune factors. IgG4-related sclerosing cholangitis (IgG4-SC) is a common extrapancreatic manifestation of AIP, however, its optimal treatment still needs to be clarified. Aims: To summarize the experience in the clinical diagnosis and treatment of AIP, especially IgG4-SC. Methods: Twenty-five patients with AIP admitted from the First Affiliated Hospital of Nanchang University between January 2015 and May 2021 were recruited, and a retrospective analysis was conducted on the clinical symptoms, laboratory and imaging findings, pathological results, as well as the treatment regimens and clinical outcomes. Results: All the patients were diagnosed as type 1 AIP (84.0% male, mean age 63.5 years). Obstructive jaundice (56.0%) and epigastric pain (44.0%) were the main clinical symptoms. The main imaging findings were diffuse enlargement of the pancreas, capsule-like rim, and distal biliary stricture associated with intra- and extra-hepatic duct dilation. The serum IgG4 elevated significantly. In 14 patients with IgG4-SC, the total bilirubin was between 1-2 upper limit of normal (ULN) in 3 cases, between 2-5 ULN in 3 cases, and > 5×ULN in 8 cases. Fifteen (83.3%) patients obtained pathological diagnosis by endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA). Six IgG4-SC patients were treated with steroids alone, and 6 received biliary stenting with or without steroids, no differences in liver function tests were existed between the two groups prior to treatment (all P> 0.05). Liver function tests in most of these patients were normalized after treatment. Conclusions: AIP is a rare disease in clinical practice, and is more frequently seen in elderly male patients. Biliary involvement (IgG4-SC) is common and often presents initially as obstructive jaundice, which can be effectively managed with steroids alone, without the need for biliary stenting.