Hepatic Sarcoidosis: Clinico-pathological characterization of symptomatic cases

The aim of this study was to investigate the clinical and pathological features of hepatic sarcoidosis in symptomatic cases.

Twenty-two symptomatic hepatic sarcoidosis cases were included in the study. Hepatic sarcoidosis was determined by typical imaging, histopathology, and high angiotensin-converting enzyme levels. Demographic data, laboratory data, imaging findings, liver biopsies, and clinical findings were analyzed. Portal hypertension (PH) was defined by the presence of ascites and/or varices; imaging findings suggestive of PH-splenomegaly (> 12 cm on longest axis); portal vein dilation (> 13 mm); collateral vessel formation; and hepatic venous pressure gradient ≥ 6 mmHg.

Mean age was 49.63 ± 10.7 years. Liver tests showed elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase levels (95%). Serum albumin levels were low (< 3 g/dl) in 32% of the patients. Histologically, hepatic granulomas were located in the portal/periportal areas, with or without parenchymal involvement (77%). Duct damage (27%), absent portal veins (32%), and hepatomegaly (41%) were also observed. Clinically, chronic cholestatic symptoms and PH features were observed in 41% and 50% of the patients, respectively. Three-quarters of patients with PH features were non-cirrhotic. Cirrhosis and bleeding varices were observed in 14%. Hepatic sarcoidosis overlaps with primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) was observed in two cases.

Sarcoidosis causes significant hepatic disease. PH and jaundice are main clinical presentations in liver sarcoidosis patients. Imaging findings of PH should be carefully reviewed, as it can occur even before the establishment of cirrhosis. Hepatic sarcoidosis mimics and overlaps with PBC and PSC.