MedStar Georgetown University Hospital Washington, DC, United States
Award: Presidential Poster Award
Camille H. Boustani, MD, Takashi Kitani, MD, Yadana Khin, BA, MS MedStar Georgetown University Hospital, Washington, DC
Introduction: COVID-19 infection leads to complications in multiple organs, including the hepato-biliary system. Secondary sclerosing cholangitis (SCC) is diagnosed when a cause for progressive cholestatic liver injury is identified in patients with no prior history of liver disease. SSC seems to stem from inflammation that can be caused by critical illness, infection, ischemia, or autoimmune disease. We present a case of severe COVID-19 infection complicated by SSC requiring liver transplantation.
Case Description/Methods: A 45-year-old male with history of type II diabetes presented to the emergency department in April 2020 with shortness of breath for 3 days and was found to be COVID-19 positive. His clinical course deteriorated, and he ultimately required ECMO for acute respiratory distress syndrome (ARDS). The patient was still recovering from his extended hospital stay, but in June he began noticing jaundice. His labs were notable for total bilirubin (11.9), direct bilirubin (8.9), AST (290), ALT (162), and alkaline phosphatase (2815). The patient underwent ERCP which revealed sludge and stones, with sphincterotomy performed and common bile duct stent placed. Beaded intrahepatic ducts with dilation consistent with sclerosing cholangitis were also noted on ERCP. Due to concern for acute cholangitis, he was started on broad spectrum antibiotics as well. The ERCP did not lead to clinical improvement, and the patient's hepatic lab abnormalities continued to worsen. An MRCP was obtained and showed diffuse dilatation and considerable beading and irregularity of the biliary tree, most compatible with an inflammatory cholangiopathy. Liver biopsy showed no evidence of chronic liver disease or primary sclerosing cholangitis but features consistent with biliary obstruction and multifocal acute cholangitis. He underwent orthotopic liver transplant in November 2020, and his MELD score was 31 at the time of surgery.
Discussion: SSC in critically ill patients (SSC-CIP) subgroup is particularly rare and was first described in 2001. Persistent cholestasis despite clinical recovery of other organ systems differentiates SSC-CIP from other hepatobiliary diseases related to critically ill patients. The pathophysiology is theorized to be due to a combination of bile duct ischemia leading to cholangiocyte necrosis and bile duct obstruction via bile cast formation. We present a case of SSC in the setting of severe COVID-19 infection requiring ECMO, resulting in liver transplantation.
Figure: MRCP showing diffuse dilation, with beading and irregularity of the biliary tree. Bile duct wall thickening and enhancement is demonstrated by the arrows.
Camille Boustani indicated no relevant financial relationships.
Takashi Kitani indicated no relevant financial relationships.
Yadana Khin indicated no relevant financial relationships.
Camille H. Boustani, MD, Takashi Kitani, MD, Yadana Khin, BA, MS. P2198 - Secondary Sclerosing Cholangitis Complicating the Course of Severe COVID-19 Infection, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.