Secondary sclerosing cholangitis after COVID-19 pneumonia: a report of two cases and review of the literature

Abstract

Secondary sclerosing cholangitis in critically ill patients (SC-CIP) is a rare disease characterized by chronic cholestasis. The underlying pathophysiology of SC-CIP is not fully understood, and prognosis in severe cases remains poor with liver transplantation remaining the only curative treatment option. There is a growing amount of literature describing patients with chronic cholangiopathy after COVID-19 infection. The vast majority of the patients described in these reports were male and had a poor outcome. While the exact percentage of patients with COVID-19-related SC-CIP cannot be estimated accurately due to a lack of larger studies, an increase in patients with long-term complications of chronic cholestatic liver disease after severe COVID19-pneumonia can be expected in the upcoming years. Treatment options remain limited and further research is needed to improve the dismal prognosis of SC-CIP. Here, we present the cases of two patients who developed SC-CIP after prolonged intensive care unit stay due to severe COVID-19 pneumonia. Both patients required invasive ventilation for 31 and 141 days, respectively, as well as extra-corporal membrane oxygenation for 23 and 87 days. The patients suffered from jaundice and severe pruritus, and typical features of SC-CIP were present by MRCP and ERC. Repeated removal of biliary casts resulted in some alleviation of their clinical symptoms, but cholestasis parameters remain elevated. Furthermore, an increased liver stiffness was indicative of advanced fibrosis in both patients. In addition to these two case reports, we provide a concise review of the literature of SC-CIP after COVID-19 infection and discuss risk factors, treatment options and prognosis.

COVID-19-Associated Liver Injury

“…The ACE-2 receptor distribution may play a role, but other causes including host inflammatory response, thrombosis, 41 bile duct ischemia, and DILI have also been postulated. 46 Interestingly, treatment with ketamine has also been suggested as a risk factor, 42 which is not necessarily uncommon given the need for sedation with intubation with severe COVID-19 disease.…”

“… 40 , 45 Although many patients with COVID-19 cholangiopathy may not be suitable candidates for transplantation due to comorbidities or degree of overall illness, cases of successful liver transplant for this indication have been reported. 42 , 45 …”

Clinical and Histopathological Discoveries in Patients with Hepatic Injury and Cholangiopathy Who Have Died of COVID-19: Insights and Opportunities for Intervention

“…Post-COVID-19 cholangiopathy is a novel entity that has been increasingly reported in the literature. [85][86][87][88][89][90][91][92][93][94] upon those basis, we conducted a scoping review of the literature, and Table 1 presents the findings as a summary of study characteristics for reports on cholangiopathy in COVID-19, with additional summaries for histopathological findings (Table 2) and MRCP/ERCP findings (Table 3). These investigations have documented various manifestations of biliary system involvement, including persistent liver dysfunction, cholestasis, and biliary abnormalities.…”

Risk of New-Onset Liver Injuries Due to COVID-19 in Preexisting Hepatic Conditions—Review of the Literature

“…There is no established protocol for SSC treatment, and the last curative option is liver transplantation [ 76 , 77 ].…”