Auto-immune cholangiopathy in a juvenile patient with systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multi-system inflammatory disease characterized by the presence of auto-antibodies. Liver enzyme abnormalities are common but clinical liver dysfunction with jaundice is rare. We report a juvenile female patient with SLE who developed jaundice 9 months after her initial presentation. Further investigations including liver biopsy and magnetic resonance cholangio-pancreatography revealed two likely pathologies for her liver dysfunction; amoxicillin-clavulanic acid induced cholestasis and auto-immune cholangiopathy. The hyperbilirubinaemia resolved spontaneously 3 months after exposure to amoxicillin-clavulanic acid; however, the elevation in Alanine transaminase and Gamma-glutamyl transpeptidase persisted until intensive immunosuppressive therapy achieved complete remission. Conclusion: We report a rare case of a juvenile patient with SLE and auto-immune cholangiopathy. The use of cholangio-pancreatography as part of the diagnostic work-up achieved the final diagnosis.