| Summary: | Autoimmune hepatitis (AIH) is an extremely rare cause of chronic liver disease (CLD) in sub-Saharan Africa—there have only been 3 reported cases of AIH in Nigeria, a country of about 200 million people. We report the first case of AIH in a male patient in Nigeria and highlight its unusual presentation. A 41-year-old man with jaundice and malaise for 3 months was referred for evaluation after investigations revealed deranged liver enzymes and a cirrhotic liver. Laboratory evaluation revealed high serum immunoglobulin G, but there was also marked elevation of serum ferritin and transferrin saturation, resulting in a diagnostic dilemma between AIH and an iron overload condition such as hemochromatosis. A liver biopsy was crucial in providing a definitive diagnosis of AIH. Despite its rarity, clinicians should maintain a high index of suspicion for AIH in sub-Saharan Africa and should proceed to a liver biopsy when the cause of CLD is unclear.
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