Cryptogenic Hepatitis: A Case Study in Autoantibody Negative Autoimmune Hepatitis

Abstract

A 76 year old white-female initially presented with signs and symptoms of acute hepatitis. While liver function tests were elevated, viral hepatitis and autoimmune hepatitis panels were negative. The patient was subsequently discharged on the 4^th day of hospitalization when her condition appeared to stabilize. Four weeks later she was readmitted to the hospital, this time with signs and symptoms of acute liver failure with ascites, confusion, and jaundice. A liver biopsy was performed with pathology suggestive of an autoimmune etiology despite the negative autoimmune antibody panel. She was diagnosed with cryptogenic autoimmune hepatitis. The patient was placed on intravenous steroids and later oral prednisone that resulted in the resolution of the liver failure and disappearance of the encephalopathy along with a downward trend of the liver enzymes, bilirubin and the prothrombin time. One year later, the liver enzymes had normalized and the autoimmune disease remained in remission with maintenance medications consisting of low dose prednisone and azathioprine.

ABBREVIATIONS: AAA - anti-actin antibody; AIH - Autoimmune Hepatitis; ALC-1 - anti-liver cytosol-1; ALT- alanine transaminase; ALP- alkaline phosphatase; AMA - antimitochondrial antibody; ANA - antinuclear antibody; ASMA - anti-smooth muscle antibody; AST - aspartate transferase; CBC - complete blood count; COPD - Chronic Obstructive Pulmonary Disease; H&E - hematoxylin and eosin; IIF - indirect immunofluorescence; LKM-1 - liver/kidney microsomal antibody Type-1; pANCA - perinuclear antineutrophil cytoplasmic antibody; PT - prothrombin time.