CASE PRESENTATION
A 27-years old Southeast Asian female patient was admitted on 24/02/2021 with complaints of fever and yellowish discoloration for 2 months which were also associated with nausea, vomiting, constipation, and black stools. She had a medical history of asthma. The patient was non-alcoholic and was neither taking any prescription nor any non-prescription medications. Also, she had a negative travel history to areas endemic for hepatitis viruses and a negative family history of any liver disease. On clinical examination, fever, hepatosplenomegaly (HSM), and jaundice were noted. The initial laboratory examinations were remarkable for hepatic injury with LFT revealing Aspartate aminotransferase (AST) 2131 IU/L, Alanine aminotransferase (ALT) 1813 IU/L, Lactate dehydrogenase (LDH) 990 IU/L, Alkaline phosphatase (ALP) 263 U/L, Gamma- Glutamyltranspeptidase (GGT) 138 U/L, Albumin 3.33 g/dl, Prothrombin time/International normalized ratio (PT/INR) and total and direct bilirubin 6.96 mg/dl and 4.68 mg/dl respectively. USG revealed mildly altered echotexture of the liver with smooth borders, mild HSM with a liver span of 16 cm in the mid-clavicular line, and spleen size of 14 cm with pericholecystic edema and mild pelvic ascites. (Figure 1)
We ruled out acute viral hepatitis due to negative Anti-HAV Ab, HBsAg, Anti-HCV Ab, and Anti-HEV Ab. There was no Kayser-Fleischer ring seen on slit lamp examination. Now, although the entire picture was pointing towards autoimmune hepatitis, but serology was negative for the classic autoantibodies including ANA (via immunofluorescences), Anti-SM Ab, Anti-LKM-1 Ab, and also a negative Immuno 17 report. This raised suspicion of seronegative AIH. The patient was also admitted on 29/01/2021 with similar complaints of pyrexia of unknown origin and jaundice but responded to symptomatic treatment. However, she relapsed now. Due to a high clinical suspicion, the patient was advised to have a liver biopsy to confirm the diagnosis, but the patient’s unstable condition didn’t permit a biopsy. Still, she was diagnosed with seronegative AIH due to her clinical picture and prescribed methylprednisone (MPS) to which the patient responded with a visible improvement of her LFT as mentioned in Table 1.
This improvement in the clinical picture following steroids’ administration confirmed the diagnosis of seronegative AIH in our patient.