Natural history of hepatic sarcoidosis and its response to treatment

BACKGROUNDLiver involvement in sarcoidosis is variable and can occur in the absence of pulmonary disease. Data on the natural history of hepatic sarcoid and response to therapy are lacking. This study investigates hepatic dysfunction complicating lung disease and significant liver involvement presenting independent of pulmonary sarcoid. PATIENTS AND METHODSOne hundred and eighty patients were included in the study. The minimum follow-up was 2 years. RESULTSFifty per cent of the study population had derangement of liver function attributable to hepatic sarcoid. Twenty-three patients (13%) had liver involvement without lung disease. Sixty-three patients were administered corticosteroids; approximately one-third had a complete clinical response, one-third a partial response and one-third showed no response. Fourteen patients (8%) were cirrhotic at presentation, and two progressed to cirrhosis despite steroid therapy. Sixteen patients received a second-line immunosuppressive agent; one-half of these showed a response to treatment augmentation (four patients azathioprine, three patients methotrexate, one patient both drugs). Six patients required liver transplantation, with disease recurrence in one recipient. In four patients, sarcoid as the aetiology of end-stage liver disease was diagnosed only on examination of the explanted liver. CONCLUSIONSarcoidosis can cause end-stage chronic liver disease, which is often unrecognized until examination of the explanted liver. Response to conventional immunosuppression is variable and unpredictable. Transplantation is feasible and safe in this population but recurrence is possible.