AUTOIMMUNE CHOLANGIOPATHY-A RARE CASE OF AN AUTOIMMUNE LIVER DISEASE

Autoimmune liver disease(AILD) is one of the rare cause of chronic liver disease in India with a prevalence of about 3.5 to 6.18 percentage among the chronic liver disease cases.Among the AILD the most commonly reported is autoimmune hepatitis,Primary biliary cirrhosis(PBC) is reported rarely.90 percentage of PBC cases will have AMA (Antimitochondrial antibody) positivity.10 percentage of the PBC cases present with negative serum AMA which is known as autoimmune cholangiopathy.We present one such case of an AMA negative PBC. An young female presented with cholestatic jaundice with negative virological markers and negative autoantibodies(AMA,ANA,ASMA) and normal imaging but liver biopsy showed features consistent with PBC histologically.She was diagnosed as autoimmune cholangiopathy and treated with ursodeoxycholicacid(UDCA) and steroids with improvement during follow up.