Beads-on-a-String Are Not Always What They Seem: A Rare Case of Sclerosing Cholangitis

Abstract

42-year-old man presented for follow-up after hospitalization for abdominal pain. His medical history included sarcoidosis and chronic obstructive hepatopathy believed to be from Primary Sclerosing Cholangitis (PSC). His PSC had been complicated by biliary strictures with failed stents and recurrent pancreatitis. He has had multiple recent admissions for abdominal pain due to pancreatitis. Since his last hospitalization, he had persistent pain, 4-5 in severity. He reported mild pruritus, a stable appetite and weight, no nausea/vomiting, no melena/hematochezia. Past surgical history includes cholecystectomy, pylorus-sparing Whipple with excision of the extrahepatic bile ducts with hepatico-jejunal reconstruction. Surgical pathology revealed a benign stricture with granulomatous inflammation. A colonoscopy 2 years prior showed no evidence of inflammatory bowel disease (IBD). He has no family history of biliary disease, IBD or gastrointestinal malignancy. His exam was normal. Labs were historically notable for negative anti-nuclear antibody, normal total IgG and subclasses within normal limits. MRCP showed a beaded appearance to the intrahepatic bile ducts with mild dilation. Main pancreatic duct (PD) stump dilated to 7 mm to the site of the pancreaticojejunostomy. Referral was made for ERCP and PD stent placement, which was unsuccessful. An interdisciplinary plan called for elective surgical pancreaticojejunostomy, distal pancreatectomy, and splenectomy. Post-operative hospital course was free of major unexpected complications and the patient recovered well. Hepatic involvement of sarcoidosis is common and it's features variable, patients may be asymptomatic. In our case, the granulomatous inflammation on surgical pathology was presumably secondary to bile duct involvement from sarcoidosis. The biliary strictures in this patient were previously believed to be related to co-existent PSC. This scenario is unlikely given the negative serology, lack of IBD and presence of sarcoidosis. In rare cases of sarcoidosis, bile duct damage, including loss of intrahepatic bile duct and ductopenia, may histologically resemble PSC. We present the case because it is a opportunity to bring to light a rare cause of secondary sclerosing cholangitis (SSC), sarcoidosis. SSC should be considered even in light of imaging/histologic findings that may be classic for PSC.Figure: MRCP prior to Whipple procedure, showing mild dilatation of the intrahepatic bile ducts in a beaded pattern.Figure: ERCP prior to Whipple procedure showing beaded appearance of the biliary tree.Figure. Beaded: appearance of intrahepatic bile ducts with mildly dilated intrahepatic ducts in the inferior right hepatic lobe. Findings consistent with PSC. Confluence not visualized. Remaining main pancreatic duct diffusely dilated to 7 mm to the site of the pancreaticojejunostomy.