Riverside Methodist Hospital Columbus, OH, United States
Veronika Kholodovych, MD, Jay Anderson, DO Riverside Methodist Hospital, Columbus, OH
Introduction: The etiology of biliary strictures requires an algorithmic evaluation: laboratory evaluation, abdominal imaging, ERCP. If etiology is not identified, the stricture is deemed indeterminate, and ruling out malignancy is imperative.
Case Description/Methods: A 34-year-old male with no past medical history presented with epigastric pain and jaundice. Laboratory analysis demonstrated an obstructive pattern, with total bilirubin 10.1, alkaline phosphatase 178, AST 106 and ALT 265. CT revealed intra and extrahepatic biliary dilation, with an indeterminate density in the distal common bile duct. EUS revealed severe biliary stricture at the pancreatic head, no mass, and diffusely hypoechoic pancreas. MRI confirmed a filling defect in the distal common bile duct. Serology displayed elevated IgG4 of 182. Index of suspicion was high for autoimmune pancreatitis, and while pathology was pending, he was discharged on a steroid regimen. Pathology then revealed benign pancreatic parenchyma, with negative immunostaining for IgG4. One month later, outpatient GI evaluation prompted the need for ERCP with additional stent placement. Biopsies revealed duodenal type mucosa with mildly increased inflammatory cells in the ampulla, and benign pancreatic parenchyma in the bile duct. The following month, repeat ERCP revealed a new left hepatic duct stricture, not amenable to dilation. Two biopsies were obtained, a biliary drain was placed, and he was recommended to continue steroids. Pathology from the left hepatic duct was benign, while pathology from the common bile duct revealed poorly differentiated adenocarcinoma.
Discussion: Biliary strictures are broadly classified as benign or malignant, and may pose a diagnostic challenge. When autoimmune pancreatitis is suspected, the diagnosis is clinically established using with HISORt criteria - histology, imaging, serology, other organ involvement, and response to therapy. This patient had no evidence of extrapancreatic involvement, and while other organ involvement supports the diagnosis, the absence thereof does not rule out the disease. While imaging raised the index of suspicion, multiple tissue samples did not reveal lymphoplasmocytic infiltrate, storiform fibrosis, or obliterative thrombophlebitis. Autoimmune pancreatitis typically responds to steroids, yet his obstruction persisted. With inconclusive pathology and lack of response to therapy suggesting an alternative diagnosis, continued pursuit of tissue was integral.
Figure: MRI sequence featuring distended gallbladder and common bile duct dilation with common bile duct tapering abruptly at level of pancreatic head.
Disclosures: Veronika Kholodovych indicated no relevant financial relationships. Jay Anderson indicated no relevant financial relationships.
Veronika Kholodovych, MD, Jay Anderson, DO. P2168 - Clinical Conundrum in a Young Male With Severe Biliary Obstruction, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.