Hepatic sarcoidosis resembling primary sclerosing cholangitis

Abstract

We report the case of a 29-year-old man who presented with progressive weight loss, night sweats, abdominal pain and pruritus who was found to have obstructive jaundice and cholestatic pattern of liver injury on laboratory workup. Though findings on magnetic resonance cholangiopancreatography were initially concerning primary sclerosing cholangitis, he was ultimately diagnosed with biliary sarcoidosis after a liver biopsy. This case brings attention to the rare phenomenon of hepatic sarcoidosis causing hyperbilirubinemia and highlights the importance of reaching the correct diagnosis early, as the patient's symptoms improved after initiation of steroids.

Keywords: gastrointestinal system; liver disease; pancreas and biliary tract.

Figure 1

Peripheral intrahepatic biliary ductal strictures and dilatation concerning for changes of PSC. There is no extrahepatic biliary dilatation. No focal hepatic lesions. Liver shows normal portal vein and hepatic vein enhancement.

Figure 2

(A) This is a representative picture of the liver biopsy, demonstrating a granulomatous nodule forming in a background of otherwise unremarkable hepatic parenchyma. The granuloma consists of an aggregate of histiocytes (cells with eosinophilic cytoplasm and elongated nuclei) and giant cells (large eosinophilic cells with numerous nuclei arranged in crescent shape). This finding is characteristic, while not specific, for sarcoidosis. (H&E stain, 100× magnification). (B) The representative picture of the portal tract of the liver biopsy shows a mild ductular reaction (bile duct proliferation) and mild lymphocytic infiltration of the portal tract. There is a lack of inflammation of the bile duct, however, as the lymphocytes are not infiltrating the ductal epithelium. Moreover, the characteristic periductal fibrosis (onion-skinning) typically seen in primary sclerosing cholangitis is not observed in the entire biopsy. (H&E stain, 100× magnification).