Type 1 (IgG4‐related) autoimmune pancreatitis: Experiences in a medical center in southern Taiwan within the past 10 years

Abstract

SummaryBackgroundsAutoimmune pancreatitis (AIP) is a relatively rare disease and has similar presentations to pancreatic cancer. There are limited reports about this disease in Taiwan.MethodsWe retrospectively reviewed 14 cases of type 1 AIP from March 2007 to March 2016 in Kaohsiung Veterans General Hospital. The diagnosis was made according to the international consensus diagnostic criteria (ICDC) for AIP.ResultsThere were 12 male and two female patients diagnosed with type 1 AIP, with a mean age of 63.6 (33 – 88) years. The rates of DM and HTN were 28.5% and 35.7%, respectively. The clinical presentations were mainly weight loss, epigastric pain, and obstructive jaundice. Most patients didn't have the habits of cigarette smoking or drinking alcohol. Minority of these patients had leukocytosis but the majority of them had anemia. All patients had a normal range of platelet count. Most patients had elevation of alanine transaminase, alkaline phosphatase or lipase. Serum immunoglobulin G (IgG) or IgG4 tests were done in most cases, and high‐up levels were seen in most of them. Six of them had an upraised CA 19‐9. All patients had a normal range of coagulation profiles (prothrombin time and activated partial thromboplastin time). Diffuse or focal swellings of the pancreas were observed from CT or MRI among different patients. Six of them had pathology evidence. Three of them received Whipple's operation. Seven of them underwent ERCP. Four of them received PTCD. Two of them received PTGBD. Corticosteroids were prescribed to all patients after the diagnosis of AIP except of that one lost to follow‐up. There were eight patients taking steroid only and five taking steroid in combination with another immunomodulator.ConclusionsType 1 AIP has several common features with pancreatic cancer so accurate diagnosis is important to avoid unnecessary operation. The diagnosis involves clinician's alertness and several diagnostic modalities, including serology, radiology and pathology. Though steroid is effective for type 1 AIP, the relapse rate is high. longterm follow‐up is required. Several clinical features in this study are different from those in other reports and further study is still required for investigation.Copyright © 2017, The Gastroenterological Society of Taiwan, The Digestive Endoscopy Society of Taiwan and Taiwan Association for the Study of the Liver.