Table 3 Clinical characteristics of patients suspected of AIP but who did not meet diagnostic criteria
Patient characteristic | PSC and elevated IgG4 | Radiological changes c/w AIP |
|---|---|---|
Number of patients | 4 | 5 |
Males (n, %) | 4 (100%) | 4 (80%) |
Age (years, mean) | 63 | 54 |
IgG4 (g/L, median) | 7.3 | NA |
IgG4 > 2 × ULN (n, %) | 4 | |
Biochemistry | ||
Bilirubin (μmol/L*) | 11.0 (11–124) | 47.0 8–217) |
ALP (U/L*) | 330.0 (71–1500) | 204.0 (100–303) |
ALT (U/L*) | 62.0 (23–234) | 91.0 (29–115) |
AST (U/L*) | 54.5 (17–229) | 41.5 (21–94) |
Presentation | ||
Obstructive jaundice | 1 | 4 |
Weight loss | 2 | 4 |
Abdominal pain | 2 | 5 |
Imaging modality | ||
CT (n, %) | 3 | 5 |
MRCP (n, %) | 4 | 5 |
ERCP (n, %) | 2 | 4 |
Radiological pancreatic parenchyma typical | ||
Diffuse | 0 | 4 |
Multifocal | 0 | 0 |
Peripheral rim enhancement | 1 | 4 |
(Halo) | 1 | 1 |
Atypical | ||
Focal | 0 | 0 |
Atrophic | ||
AIP Radiological classification | ||
SC | 3 | 0 |
SP-SC | 0 | 3 |
SP | 0 | 2 |
Biliary changes | ||
Intrahepatic duct | 4 | 2 |
Proximal extra-hepatic duct | 2 | 4 |
Initial treatment | ||
Steroids | 3 | 0 |
ERCP and biliary stenting | 0 | 4 |
Steroids and biliary stenting | 0 | 0 |
Response to steroids | 1 | 0 |
Complications | ||
Cholangiocarcinoma | 1 | 0 |