Sarcoidosis is a rare inflammatory disease characterized by clusters of white blood cells, called granulomas, which may form in any organ or tissue. These granulomas are damaging, and can affect the proper functioning of an involved organ.

When granulomas form in the liver, a patient is diagnosed with hepatic sarcoidosis. Any part of the liver can be affected.

Estimates of total cases of sarcoidosis affecting the liver vary widely, ranging an extreme low of 6 percent to almost 80 percent. The lower rates are usually based on symptomatic disease; estimations based on liver biopsies tend to report higher rates, usually 50 to 80 percent. But many patients are asymptomatic, despite granulomas detected in a biopsy or findings of abnormal liver enzymes.

Symptoms of hepatic sarcoidosis

Hepatic sarcoidosis may cause an increase in liver enzymes and cholestasis, a reduction in bile flow. But, as noted, evidence of severe liver damage and organ dysfunction is rare and most patients show no disease symptoms.

When symptoms occur, they include jaundice (a yellow skin), itching skin, and pain in the right upper quadrant of the abdomen where the liver is situated. High blood pressure frequently develops in patients with long-standing disease.

Symptoms less specific to the condition are fatigue, fever, and arthralgia or joint pain.

Diagnosis of hepatic sarcoidosis

Hepatic sarcoidosis is difficult to diagnose because the symptoms are usually not specific to liver disease. Blood tests may help in attaining a diagnosis.

Damage or inflammation of liver cells caused by sarcoidosis can lead to an increase in liver enzymes in the blood. High levels of two such enzymes, called alkaline phosphatase and gamma-glutamyltranspeptidase, are usually associated with hepatic sarcoidosis.

Angiotensin-converting enzyme (ACE) levels are also elevated in many sarcoidosis patients, but this is not specific to patients with hepatic sarcoidosis. ACE is produced by epithelial cells and macrophages (a type of immune cell) of the granuloma. Normal ACE levels do not exclude sarcoidosis, but help distinguish sarcoidosis from other granulomatous liver diseases.

Sarcoid granuloma clusters in the liver can be detected through imaging techniques such as ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI). But it can be difficult to distinguish sarcoidosis clusters from other granulomatous diseases, and if multiple clusters are present, they might be mistaken for liver tumors.

A liver biopsy may be necessary to confirm a diagnosis of hepatic sarcoidosis.

Treatment of hepatic sarcoidosis

Most hepatic sarcoidosis cases are non-symptomatic and do not require treatment. However, they need to be carefully monitored using liver function tests to rule out the development of cholestasis.

In symptomatic patients, corticosteroids, such as prednisone and ursodeoxycholic acid, a bile acid, are most commonly used to treat the condition. Corticosteroids suppress the inflammatory response, working to reduce the number of hepatic granulomas and liver size. Ursodeoxycholic acid is widely used to treat cholestasis. Several studies have shown it can be effective in hepatic sarcoidosis patients with cholestasis.

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