Internal Medicine Hospitalist Rush University Medical Center Chicago, IL
Muhammad Alsayid, MD, MPH, Anas Alabkaa, MD, Ajaypal Singh, MD; Rush University Medical Center, Chicago, IL
Introduction: Hepatic manifestations occur in 12.8% of patients with sarcoidosis. Enlarged lymph nodes in the porta hepatis may cause bile duct obstruction and strictures. However, extrahepatic biliary strictures due to primary involvement of the bile duct is extremely rare. We present a patient with extrahepatic biliary obstruction due to sarcoidosis.
Methods: A 56-year-old African American woman presented to the ER with a 2-day history of nausea and painless jaundice. Vital signs were within normal limits and physical exam revealed icteric sclera. Laboratory showed total bilirubin of 8.4 mg/dL, conjugated bilirubin 7.5 mg/dL, alkaline phosphatase 606 IU/L, AST 107 IU/L, ALT 189 IU/L, and lipase 132 U/L. CT abdomen/pelvis with IV contrast showed moderate central intrahepatic biliary ductal dilatation with compression of the bile duct confluence secondary to enlarged lymph nodes at the porta hepatis. CA 19-9 and AFP were normal. EUS revealed intrahepatic biliary duct dilation and multiple enlarged lymph nodes in the porta hepatis. FNA/FNB were performed. ERCP showed a single severe stricture in the hepatic biliary duct bifurcation 20 mm in length. Sphincterotomy was performed and a plastic stent was placed in the dilated left system. Biopsy results showed reactive multi-nucleated giant cells without malignancy. A repeat EUS and ERCP were performed within 6 weeks and biopsies from lymph nodes showed sarcoid-like granulomas without malignancy. The patient was started on prednisone 30 mg daily for sarcoidosis once her liver enzymes were normalized. She underwent ERCP with cholangioscopy, which showed irregular and nodular mucosa of the bile duct and it was biopsied. Biopsy results showed epithelioid histiocytes forming non-caseating granulomas. Subsequently, the patient underwent multiple ERCP procedures with plastic stents exchanges along with steroids and other immunomodulators to treat the biliary stricture. Nonetheless, the bile duct stricture did not respond to treatment so hepatojejunostomy is being considered. Discussion: Extrahepatic biliary obstruction that is caused by enlarged lymph nodes in the porta hepatis improves with steroids due to the reduction is size of lymph nodes. Our patient had an improvement in the size of lymph nodes in response to steroids, however she continued to have a biliary stricture due to primary involvement of the bile duct. Therefore, hepatojejunostomy is being considered as a curative option.