PMC10184037_01
A timeline showing the disease course of this case.
tgh-08-21-111-f1.jpg
PMC10184037_01_tgh-08-21-111-f1.jpg
f1
Laboratory tests on admission showed elevated levels of total bilirubin (102.7 micromol/L, reference range, 5.1-22.2 micromol/L) (Figure 1), direct bilirubin (68.4 micromol/L, reference range, 0-8.6 micromol/L), alkaline phosphatase (953.92 U/L, reference range, 45-125 U/L), gamma-glutamyltransferase (1,407.5 U/L, reference range, 10-60 U/L), alanine aminotransferase (1,030.07 U/L, reference range, 9-50 U/L), aspartate aminotransferase (589.62 U/L, reference range, 15-40 U/L), serum amylase (192.00 U/L, reference range, 30-110 U/L), serum lipase (2,956.0 U/L, reference range, 23-300 U/L), and IgG4 (499 mg/dL, reference range, 1.2-201 mg/dL). Serum IgG4 level and liver function markers gradually recovered during follow-up (Figure 1). Except for an elevated serum IgG4 level of 388 mg/dL, laboratory tests revealed significantly elevated levels of total bilirubin (161.8 micromol/L, reference range, 5.1-22.2 micromol/L), direct bilirubin (112.9 micromol/L, reference range, 0-8.6 micromol/L), alkaline phosphatase (566.75 U/L, reference range, 45-125 U/L), gamma-glutamyltransferase (1,510.32 U/L, reference range, 10-60 U/L), alanine aminotransferase (758.53 U/L, reference range, 9-50 U/L), aspartate aminotransferase (321.94 U/L, reference range, 15-40 U/L) (Figure 1). IgG4 level and liver function normalized after a 1.5-month steroid therapy (Figure 1).
MRI findings at the time of first attack and disease relapse in this patient. (A,B) Images before steroid therapy at the first attack. (A) Diffuse enlargement of pancreas with delayed enhancement (red arrows) and rim-like enhancement; dilated intrahepatic bile duct (black arrows). (B) Circular-symmetrical wall thickening in the lower common bile duct (white arrow). (C,D) Images after steroid therapy at the first attack. (C) Mild enlargement of pancreas with delayed enhancement (red arrows). (D) Thickened lower common bile duct wall with no significant change compared to previous images (white arrow). (E,F) Images before steroid therapy at the disease relapse. (E) Swollen pancreas morphology with no significant change compared to the previous images (red arrows), dilated intrahepatic bile duct (black arrows). (F) Thickened upper segment of common bile duct wall (white arrow), dilated intrahepatic bile duct (black arrows). MRI, magnetic resonance imaging.
tgh-08-21-111-f2.jpg
PMC10184037_01_tgh-08-21-111-f2.jpg
f2
C, and E, and tumor markers (i.e., alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen-199, 50, 24-2, 125, and 153). Contrast-enhanced magnetic resonance imaging (MRI) showed diffuse enlargement of pancreas with its delayed enhancement (Figure 2). MRI and MRCP also showed the improvement of pancreatic enlargement and biliary stricture on December 3, 2020 (Figures 2,3). MRI showed a slightly swollen pancreas, which was not significantly deteriorated compared to the previous images (Figure 2).
MRCP findings at the first attack and disease relapse in this patient. (A,B) Images before (A) and after (B) steroid therapy at the first attack. (A) Dilated intrahepatic bile duct and common bile duct (white frame) with an inner diameter of about 1.3 cm, narrowed lower common bile duct (red frame), and obscure pancreatic duct (white arrows). (B) Improvement of dilated intrahepatic bile duct and common bile duct with a diameter of about 0.9 cm, improvement of narrowed low common bile duct (red frame), and clear pancreatic duct (white arrows). (C,D) Images before (C) and after (D) steroid therapy at the disease relapse. (C) Dilated intrahepatic bile duct (white frame), narrowed hilar bile duct and lower common bile duct (red frames) with a diameter of about 0.7 cm, and obscure pancreatic duct (white arrows). (D) Significant improvement of narrowed hilar bile duct and lower common bile duct (red frame), and clear pancreatic duct (white arrows). MRCP, magnetic resonance cholangiopancreatography.
tgh-08-21-111-f3.jpg
PMC10184037_01_tgh-08-21-111-f3.jpg
f3
Magnetic resonance cholangiopancreatography (MRCP) showed pancreatic duct stricture, intrahepatic bile duct and upper middle segment of common bile duct dilatation, and lower common bile duct stricture with slight wall thickness (Figure 3). MRCP showed intrahepatic bile duct dilatation as well as hilar bile duct and lower common bile duct stricture with wall thickness (Figure 3). On the 10th day of steroid therapy, MRCP showed a significant improvement of bile duct stricture (Figure 3).