RESUMO
Primary biliary cirrhosis is a rare cause of chronic liver disease in Myanmar. It usually presents with pruritus and fatigue in a middle-aged female before the appearance of jaundice. This case illustrates a woman presenting with 15 years of pruritus and 5 years of intermittent jaundice. She had no risk factors for viral hepatitis and was a non-drinker. Multiple consultations were done and treated as a chronic liver disease without probing further for the etiology. Persistently high alkaline phosphatase of biliary origin prompted us to test for anti-mitochondrial antibodies which were positive for M2 subtype. Furthermore, she was found to have clinical and echocardiographic evidence of mitral stenosis (MS). MS is usually a consequence of post-streptococcal rheumatic carditis. Molecular mimicry is the common mechanism in the pathogenesis of both conditions. Her symptoms improved after ursodeoxycholic acid treatment.