Current concepts on pathogenesis, diagnosis and management of hepatic sarcoidosis.

Sarcoidosis is an inflammatory multisystemic granulomatosis of unknown cause, with a wide range of clinical features, characterized by the presence of non-caseating granulomas. Liver is the third involved organ after lungs and lymph nodes, with a reported prevalence of hepatic involvement in 5 to 25% of systemic symptomatic sarcoidosis. The immunopathogenesis of sarcoidosis is still unknown but it seems to involve the innate and adaptive immune actors in response to a putative antigen in genetically predisposed individuals. Because of its paucisymptomatic presentation, hepatic sarcoidosis may be underdiagnosed. Unspecified impaired general condition, fever, abdominal pain, and jaundice are the main symptoms associated with liver sarcoidosis. Frequently, laboratory liver tests are abnormal. Imaging tools may reveal liver nodular enlargement but can be totally normal. Liver biopsy is often required to confirm the diagnosis. A meticulous workup is mandatory to rule out differential diagnosis of hepatic granuloma. Portal hypertension and liver cirrhosis are the most prevalent complications of hepatic sarcoidosis. Treatment is not necessary in all cases and first line treatment in symptomatic patients requires corticosteroids and/or ursodeoxycholic acid. Immunosuppressants and biologics could be used as second line agents. In severe cases, liver transplantation is indicated.