Hepatopulmonary Syndrome and Liver Sarcoidosis: A Tale of Two Diseases

Abstract

Hepatopulmonary syndrome (HSP) is characterized by the triad of advanced liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. Most cases of HSP are associated with cirrhotic portal hypertension; however, it has also been reported in acute liver failure patients. An estimated prevalence of HPS in patients with chronic liver disease is around 5-32%. Sarcoidosis is a granulomatous disorder with noncaseating granulomas involving the lungs in 90% of cases. Sarcoidosis can involve the liver but in only about 1% of cases does it lead to cirrhosis and portal hypertension. In this review, we report a rare case of liver cirrhosis due to sarcoidosis with associated hepatopulmonary syndrome and discuss this in detail.

sarcoidosis and digesTiVe sysTem organs inVol VemenT

“…Саркоидоз печени только в 1% случаев приводит к циррозу и пор-тальной гипертензии, отмечены лишь отдельные случаи ГПС, вызванные саркоидозом [58].…”

Hepatopulmonary syndrome caused by sarcoidosis of the liver treated with transjugular intrahepatic portosystemic shunt

“…Taken together with the other findings, severe HPS due to sarcoid liver disease is the most likely diagnosis. I have found only three reported cases with sarcoidosis of the liver causing HPS 3. All patients were initially diagnosed as having extrahepatic sarcoidosis and developed serious affection of the liver with granulomas and cirrhosis.…”