key: cord-0031666-z8k3zecg authors: Guri, Yakir; Vosbeck, Jürg; Dickenmann, Michael; Jetter, Alexander; Bernsmeier, Christine title: Exacerbation of Familial Intrahepatic Cholestasis in Conjunction with COVID-19 Vaccination date: 2022-05-16 journal: J Hepatol DOI: 10.1016/j.jhep.2022.05.003 sha: 5a94b0addebf90c813665941bb17daf85737efaa doc_id: 31666 cord_uid: z8k3zecg nan The highly transmissible Severe Acute Respiratory Coronavirus 2 (SARS-CoV-2) leading to coronavirus disease 2019 (COVID-19) is imposing a devastating global impact, leading to the fast development of efficient anti-COVID-19 vaccines. These may rarely impose side effects including autoimmune hepatitis (1, 2) . Here, we report the case of a 53-year-old man presenting in our emergency department with jaundice and pruritus. Within 48 hours following BTN162b2 Pfizer-BioNTech mRNA COVID-19 vaccination he felt fatigued, developed nausea, severe ubiquitous pruritus and a temperature (37.5-38°C). Progressive jaundice occurred within 72h post vaccination. He The typical temporal relationship in the absence of other possible triggers suggests that BRIC exacerbation in this patient occurred likely due to the COVID-19 vaccination. Yet, a direct causality cannot be definitively established. This is, to our knowledge, the first report of BRIC exacerbation developing post COVID-19 vaccination. The WHO database on adverse drug effects has reported no case of PFIC in combination with a COVID-19 vaccination to date (04 APR 2022, BRIC is not a search term). The underlying mechanism remains unclear, it is possible that immune responses and cytokine release triggered cholestasis similar to immune responses following infection. Previously, BRIC exacerbation was reported to occur following hepatitis A vaccination (4) . We do not intend to discourage COVID vaccination. Nonetheless, this severe manifestation of cholestasis complicated by AKI requiring intensive care in a patient with BRIC1 related to a homozygous ATP8B1 variant should arouse our awareness for heretofore unknown hepatic responses to the COVID vaccine and the consideration of a possible harm benefit potential in this patient group. Legend Figure 1 A. The levels of bilirubin, bile acids and creatinine over the course of the BRIC exacerbation. On the x axis depicted is the time in months from the onset of BRIC exacerbation; an asterisk J o u r n a l P r e -p r o o f Liver injury following SARS-CoV-2 vaccination: A multicenter case series Immune-mediated hepatitis with the Moderna vaccine, no longer a coincidence but confirmed Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency Benign recurrent intrahepatic cholestasis: review and long-term follow-up of five cases We acknowledge the whole patients' family who participated in the genetic family testing.Moreover, we acknowledge Julia Laube and Dr. Markus Zweier from the Institute of Medical Genetics at the University of Zurich for performing the sequencing analyses.