Newly developed autoimmune cholangitis without relapse of autoimmune pancreatitis after discontinuing prednisolone

Abstract

A 57-year-old man presented with a 2-wk history of painless jaundice and weight loss. He had a large ill-defined enhancing mass-like lesion in the uncinate process of the pancreas with stricture of the distal common bile duct. Aspiration cytology of the pancreatic mass demonstrated inflammatory cells without evidence of malignancy. Total serum immunoglobulin G level was slightly elevated, but IgG4 level was normal. After the 2-wk 40 mg prednisolone trial, the patient's symptoms and bilirubin level improved significantly. A follow-up computed tomography (CT) scan showed a dramatic resolution of the pancreatic lesion. A low dose steroid was continued. After six months he self-discontinued prednisolone for 3 wk, and was presented with jaundice again. A CT scan showed newly developed intrahepatic biliary dilatation and marked concentric wall thickening of the common hepatic duct and the proximal common bile duct without pancreatic aggravation. The patient's IgG4 level was elevated to 2.51 g/L. Prednisolone was started again, after which his serum bilirubin level became normal and the thickening of the bile duct was resolved. This case suggests that autoimmune pancreatitis can progress to other organs that are not involved at the initial diagnosis, even with sustained pancreatic remission.

Keywords: Autoimmune disease; Cholangitis; Pancreatitis; Prednisolone.

Figure 1

Initial abdominal computed tomography scan revealed an ill-defined enhancing mass-like lesion in the uncinate process of the pancreas with a dilatation of common bile duct. A: The uncinate process of the pancreas (white arrow) measuring 5.7 cm × 3.2 cm with regional infiltrations; B: In addition to this, there is small amounts of fluid collections around the pancreas head (white arrows).

Figure 2

Magentic resonance cholangiopancreatography and endoscopic retrograde cholangiography. A: Magentic resonance cholangiopancreatography showed moderate dilatation of the intrahepatic and common bile duct. The distal common bile duct had an abrupt narrowing. The pancreatic duct was unremarkable; B: Endoscopic retrograde cholangiography revealed a beak shaped stricture of the distal common bile duct with biliary dilatation above it.

Figure 3

Photomicrograph of cytologic specimen obtained by endoscopic ultrasound-fine needle aspiration, showing lymphocytes (arrows) and irregular sheets of bland ductal epithelial cells on the bloody background (hematoxylin and eosin, × 400).

Figure 4

Follow up abdominal computed tomography scan. A: Coronal image revealed a stenosis of the common hepatic and the proximal common bile duct (white arrow) with significant thickening and inner wall enhancement of the bile duct; B: There was no pancreatic relapse (white arrow).

Figure 5

Abdominal computed tomography scan after retreatment with prednisolone showed resolution of the thickening of the bile duct (white arrow).