Diagnosis and treatment of hepatic sarcoidosis

Abstract

The presence of granulomas in the liver raises consideration of a wide differential diagnosis, but in most Western series, sarcoidosis accounts for a majority of cases. This review will focus specifically on the diagnosis of and therapy for hepatic sarcoidosis. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Hepatic involvement of sarcoidosis was described in 11.5% of 736 patients enrolled in the ACCESS study. However, presence alone of granulomas in an organ in sarcoidosis does not dictate treatment. The decision to treat should be based on symptoms and severity of disease. Although hepatic involvement usually is asymptomatic, a minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may require liver transplantation. Treatment of hepatic sarcoidosis should be reserved for patients who manifest this spectrum of disease. Glucocorticoid treatment is first-line therapy for hepatic sarcoidosis, improving symptoms and abnormal laboratory values but generally having no effect on progression of disease. In addition to glucocorticoids, immunomodulators such as azathioprine, methotrexate, hydroxychloroquine, and infliximab have been used with some positive effects on symptoms, liver enzyme abnormalities, and hepatomegaly, but none has been shown to prevent progression of disease. Ultimately, in cases of overt liver failure, liver transplantation is the definitive treatment. Overall, treatment for hepatic sarcoidosis is targeted toward alleviation of symptoms but has no curative potential at this time. Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.

Liver-test abnormalities in sarcoidosis

“…Extrapulmonary sites, including the liver, are involved in 50% of the cases [4,6,7]. Hepatic sarcoidosis mostly affects young individuals between 20 and 40 years old, and is twice as common in African-Americans as in Caucasians [6][7][8][9][10][11][12].…”

“…Hepatic sarcoidosis mostly affects young individuals between 20 and 40 years old, and is twice as common in African-Americans as in Caucasians [6][7][8][9][10][11][12]. Hepatic sarcoidosis is usually asymptomatic [4,8,13].…”

Sarcoidosis-Lymphoma Syndrome presenting by severe Cholestatic Hepatitis

“…The fi nding of sarcoid granulomas in the liver is frequent; however, severe hepatic affection is rare [5].…”

“…Liver affection in sarcoidosis has been variably reported at rates of 11 to 80%, mostly in the form of elevated serum alkaline phosphatase and gamma glutamyl transpeptidase [4]. Clinically evident liver dysfunction occurs at lower rates, even in those with numerous hepatic granulomas [5]. In patients with sarcoidosis, cholestatic hepatitis is rarely seen [6].…”

Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases?

“…11,12 Certain features help distinguish granulomas in PBC from those associated with sarcoidal disease. The granulomas in sarcoidosis tend to be multiple and may coalesce into large groups.…”