Sarcoidosis is a multisystem inflammatory disorder characterized by the presence of granulomas in lymph nodes, lungs, and other organs. The liver is among the most common extrapulmonary sites compromised by the disease, and affected individuals are diagnosed with hepatic sarcoidosis.
Up to 70% of patients suffering from HS are asymptomatic , while a majority of those who develop symptoms claim abdominal pain. In this context, an abdominal examination may reveal hepatomegaly and possibly splenomegaly . Constitutive symptoms like fatigue, fever, and arthralgia have occasionally been reported. Few patients present with cholestasis and consequent pruritus, jaundice, pale stools and dark urine. Others suffer from portal hypertension. The latter commonly results from biliary cirrhosis and may lead to ascites, peripheral edema, and right heart failure . Similarly, portal hypertension open link may be caused by the formation of thrombi in hepatic veins, a condition known as Budd-Chiari syndrome .
Of note, sarcoidosis is a systemic disease rarely limited to the liver. Thus, patients may present with lymphadenopathy, pulmonary sarcoidosis, skin lesions, uveitis and neurological deficits, among others. In fact, the presence of symptoms not consistent with hepatic disease is of major importance to diagnose HS.
Elevated serum concentrations of alkaline phosphatase and γ-glutamyltransferase are characteristic of but not specific for HS. Indeed, the incidental detection of such anomalies is often the first hint at the disease. They may or may not be accompanied by increases in alanine aminotransferase and aspartate aminotransferase concentrations. Furthermore, enhanced levels of serum angiotensin-converting enzyme have repeatedly been measured in samples obtained from HS patients.
Foci of granulomatous inflammation may be visualized applying sonography, computed tomography, or magnetic resonance imaging  . Granulomas typically depict as hypointense or hypodense areas and often concentrate in periportal regions. Granulomas are usually observed in lymph nodes and other end-organs, too . Because isolated HS is very rare , the presence of granulomas in non-hepatic tissues is sometimes considered a prerequisite for the diagnosis of HS.
Based on blood tests and diagnostic imaging alone, it may be difficult to distinguish HS from tuberculosis. In order to do so, biopsy specimens have to be obtained and examined for the presence of acid-fast bacilli. Additionally, histopathological analyses of such samples allow to rule out malignancies. HS-related granulomas are non-caseating, epithelioid granulomas.
Sarcoidosis is an inflammatory disease of unknown etiology that may comprise distinct organ systems. While pulmonary sarcoidosis is most frequently observed, up to 70% of sarcoidosis patients present with non-caseating granulomas in hepatic tissues . This condition is referred to as hepatic sarcoidosis (HS). Affected individuals are mostly asymptomatic, but may develop cholestasis and portal hypertension. To date, there is no cure for HS and patients are most commonly prescribed anti-inflammatory drugs to limit disease progression  . While HS is generally associated with a favorable prognosis, liver transplantation may become necessary in severe cases . Otherwise, sarcoidosis-related morbidity and mortality mainly result from pulmonary, cardiac and central nervous system involvement .