Secondary sclerosing cholangitis in critically ill patients: a rare disease precipitated by severe SARS-CoV-2 infection

Abstract

A previously well 59-year-old man required a prolonged intensive care unit stay due to severe COVID-19 symptoms. During the admission, he developed a cytokine storm, also known as secondary haemophagocytic lymphohistocytosis, and multiorgan failure. Despite recovering from his other organ failures, his liver function continued to deteriorate. Magnetic resonance cholangiopancreatography and subsequent endoscopic retrograde cholangiopancreatography revealed extensive intrahepatic duct dilatation with 'beading' but common bile duct sparing. Given the patient had no primary liver disease prior to admission, we considered secondary causes of cholestatic liver injury; this led us to an unusual diagnosis of secondary sclerosing cholangitis in critically ill patients. This case demonstrates a rare disease that has developed specifically in the context of SARS-CoV-2 infection. A review of current literature and the underlying pathophysiology for this rare disease are discussed, particularly in relation to COVID-19.

Keywords: adult intensive care; infectious diseases; liver disease.

Figure 1

Cumulative figures of serum bilirubin, alanine transaminase (ALT) and alkaline phosphatase (ALP) concentrations throughout the patient’s secondary care admission. The green vertical line demonstrates discharge from intensive care unit (ICU), and the red vertical line demonstrates discharge from secondary care.

Figure 2

Magnetic resonance cholangiopancreatography image showing ‘beading’ effect of intrahepatic bile ducts.

Figure 3

Cholangiogram taken during endoscopic retrograde cholangiopancreatography revealing a sclerosing cholangitis type picture within the intrahepatic ducts.