key: cord-0871609-fra9m6tv authors: Zhou, Taotao; Fronhoffs, Florian; Dold, Leona; Strassburg, Christian P.; Weismüller, Tobias J. title: New-Onset Autoimmune Hepatitis following mRNA Covid-19 Vaccination in a 36-year-old woman with Primary Sclerosing Cholangitis – should we be more vigilant? date: 2021-08-25 journal: J Hepatol DOI: 10.1016/j.jhep.2021.08.006 sha: 364b80121d3c511b8ce8617f3377ae7b55709044 doc_id: 871609 cord_uid: fra9m6tv nan To the Editor, Recently, colleagues from different institutions reported in this journal on five cases of newly diagnosed autoimmune hepatitis (AIH) following COVID-19-vaccination with the Pfizer/BioNTech [1, 2] or the Moderna mRNA-vaccine [3] [4] [5] . The patients were all female, between 35 and 80 years of age and liver specific symptoms occurred between four [3] and 35 days [4] after the 1 st resp. 7 days after the 2 nd vaccination [2, 5] . Two patients had possibly confounding risk factors for AIH development namely recent pregnancy [1] and autoimmune thyroiditis [2] . The five cases have stirred up discussion whether mRNA vaccines could trigger hepatic autoimmune reaction through molecular mimicry or bystander activation of dormant autoreactive T-helper-cells [6] . Here we add another case to this discussion, which could possibly enlighten new aspects, as the first case of a patient with pre-existing primary sclerosing cholangitis (PSC) diagnosed with AIH after mRNA-COVID-vaccination. PSC is a chronic progressive cholestatic liver disease characterized by multi-focal biliary strictures. A subgroup of PSC-patients shows biochemical, serological, and/or histological features overlapping with those of AIH [7] and in the largest international PSC cohort study 8.1% of the 2454 female and 5.8% of the 4661 male patients were diagnosed as PSC/AIH variant [8] . Our patient (female, 36years) was diagnosed at 33years with ulcerative colitis and PSC. Diagnosis was made after exclusion of viral or metabolic hepatopathy due to a typical cholangiogram and highly elevated alkaline phosphatase (AP, 1077U/l) and gamma glutamyltransferase (GGT, 757U/l), while aspartate aminotransferase (AST, 117U/l), alanine aminotransferase (ALT, 193 U/l) and Immunoglobulin G (IgG, 16.9g/l) were only slightly elevated. Following treatment with ursodeoxycholic acid (1000mg/d) and endoscopic biliary intervention AP and gGT initially decreased and ALT and AST normalized. However, after one year, the patient presented with cholangitis and pruritus and ERC showed multiple highgrade intra-and extrahepatic strictures (suppl. Fig.1 ). Due to recurrent episodes of purulent cholangitis and refractory strictures scheduled balloon dilatations had to be performed regularly every three months. During episodes of cholangitis aminotransferases were also elevated but less than AP/GGT (Fig. 1) . Hence, we diagnosed AIH and started treatment with prednisone (50 mg) on June 10 th with rapidly decreasing liver enzymes. Since the SARS-CoV2-antibody-titer two weeks after the Autoimmune hepatitis developing after coronavirus disease 2019 (COVID-19) vaccine: Causality or casualty? Autoimmune hepatitis following SARS-CoV-2 vaccine: May not be a casualty The mRNA COVID-19 vaccine -a rare trigger of Autoimmune Hepatitis? Autoimmune hepatitis following COVID-19 Vaccination: true causality or mere association? Another case of autoimmune hepatitis after SARS-CoV-2 vaccination. Still casualty? Autoimmune hepatitis developing after coronavirus disease 2019 (COVID-19) vaccine: Causality or casualty? Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis European Association for the Study of the L. EASL Clinical Practice Guidelines: Autoimmune hepatitis