A Rare Cause of Prolonged Cholestatic Hepatitis - Hepatic Sarcoidosis

Background and Aim: Hepatic involvement in sarcoidosis is not uncommon. We describe an interesting case of hepatic ssarcoidosis with cholestatic hepatitis. Case Report: A 46-years diabetic female presented with jaundice and pruritus for four weeks. She had cholecystectomy five years back. Physical examination revealed deep icterus and mild hepatomegaly. Investigations showed total bilirubin 16 mg%, 4-6-fold elevated transaminases and 6-8-fold elevated alkaline phosphatase. Ultrasonography showed mild hepatomegaly and normal biliary system. Viral markers (Hepatitis A and E), ANA, SMA, AMA and tissue transglutaminase antibody were all negative. MRCP and endoscopic ultrasound showed normal biliary system. These investigations were repeated after 4-5 weeks and results were similar. Due to intense pruritus despite medical treatment, ERCP and external biliary drainage was done. Jaundice and pruritus improved partially within 2 weeks, but mild jaundice persisted, and alkaline phosphatase remained elevated. CT scan of chest/abdomen and PET scan showed evidence of hilar & mediastinal lymph node enlargement with changes of interstitial lung disease. Angiotensin converting enzyme level was elevated, mantoux test was negative and liver biopsy showed granulomatous inflammation consistent with sarcoidosis. Her symptoms improved with oral prednisolone, but she relapsed twice on tapering dose of prednisolone. Azathioprine was added (1.5 mg/kg) which she continued for two years and tapered off. There was no relapse over next one year. Conclusion: Sarcoidosis is systemic granulomatous disease characterised by non-caseating granulomas which most commonly occur in lungs. Hepatic involvement is not uncommon but prolonged cholestatic hepatitis is rare and is generally not considered in differential diagnosis of such syndrome. Here we are reporting rare manifestation of sarcoidosis in the form of prolonged cholestatic hepatitis.