Steroid-Responsive Seronegative Autoimmune Hepatitis (SAIH) Associated with Yellow Fever Vaccine. A Case Report

Abstract

Purpose: Acute seronegative autoimmune hepatitis is a rare entity but has been reported in the literature. Few cases have been reported where hepatitis occured after yellow fever vaccine. We present a case of acute seronegative autoimmune hepatitis (SAIH) that has probable association with yellow fever vaccine as indicated by the patient's strong history. Case: A 35-year old Caucasian lady presented to the outpatient clinic with right upper quadrant (RUQ) abdominal pain and intermittent low grade fever for 2-4 weeks. Her past medical history was significant with yellow fever vaccine one month as well as 3 years ago. Her physical examination was normal except mild jaundice. She was admitted to the hospital and her labs were significant for abnormal liver panel (AST: 2135; ALT: 2189; Alkaline Phosphatase: 213; Total Bilirubin: 2.5; Direct Bilirubin: 1.8; Total Protein: 7.9; Albumin: 3.6; PT: 13.2; INR: 1.3). CT abdomen was unremarkable for biliary system abnormalities Her viral panel (HSV, EBV, HIV, Hepatitis A-D serology) and autoimmune work-up (ANA, anti DNA, anti smooth muscle, anti mitochondrial, SSA, SSB antibodies turned) were negative. PCR for Yellow fever virus was negative as well. On the subsequent day, her liver enzymes were as follows: AST: 1087, ALT: 1146, Total Bilirubin: 20.7, Direct Bilirubin: 12.5, total protein 7.0 and albumin 2.7. No specific therapy had been started by that time. Liver biopsy showed severe chronic active hepatitis (grade III) without fibrosis (grade 0) but with extensive periportal necro-inflammatory changes, hence, a working diagnosis of SAIH was made. Patient was given a trial of steroids (1 gm of solumedrol followed by 40mg of prednisone daily from next day). She responded well to the treatment with AST: 374; ALT: 603; Alkaline Phosphatase: 132; Total Bilirubin: 19.1; Direct Bilirubin; 10.2 within a couple of days. She was discharged and followed up closely in the liver clinic. Her hepatic panel at 2 weeks showed AST: 67; ALT: 78; Alkaline Phosphatase: 83; Total Bilirubin: 3.6. We were able to taper her steroids in the next 4 months with maintenance treatment of 5mg Prednisone. She is still receiving the maintenance therapy and so far her liver panel has been stable. Conclusion: It is concluded that given the history of this patient, the possibility of association of yellow fever vaccine inducing acute flare of autoimmune hepatitis cannot be ruled out. Further studies to unearth this rare association in detail are warranted.