Hepatic sarcoidosis presenting as portal hypertension and liver cirrhosis: case report and review of the literature

Abstract

Systemic sarcoidosis is a disease of unknown etiology, with the liver being the third most commonly affected organ. Most cases of hepatic sarcoidosis are not clinically apparent, but a few can progress to liver cirrhosis, portal hypertension and ultimately liver failure. The diagnosis of hepatic sarcoidosis is difficult, considering that no single laboratory test or radiographic finding can definitively diagnose this systemic disease. Diagnosis of hepatic sarcoidosis relies heavily on histopathologic evaluation of two or more organs, a diagnostic modality that is invasive and may not be applicable to all patients. The treatment of hepatic sarcoidosis is challenging, with no large randomized controlled trials done to date. Physicians must be aware of the complications of hepatic sarcoidosis, and must include the same in the differential diagnosis of liver cirrhosis. We present a case of hepatic sarcoidosis complicated by portal hypertension and liver cirrhosis.

Keywords: Extrapulmonary sarcoidosis; Gastrointestinal sarcoidosis; Hepatic sarcoidosis; Hepatic sarcoidosis and liver cirrhosis; Hepatic sarcoidosis and portal hypertension; Liver cirrhosis; Portal hypertension.

Fig. 1

Liver biopsy revealing the presence of a non-caseating granuloma (arrow) with epitheloid cells.

Fig. 2

a Enlarged spleen with multiple nodular pattern typical of sarcoidosis. b Microscopic specimen of the spleen revealing multiple non-caseating granulomas (arrows) composed of epitheloid cells and multi-nuclear giant cells.

Fig. 3

CT scan of the abdomen. Note the diffuse heterogeneity of the liver parenchyma with diffuse nodularity of the liver contour (arrows), highly suggestive of liver cirrhosis. Mild ascites was also noted (arrowhead).