Clinical presentation and protocol for management of hepatic sarcoidosis

Abstract

The liver is one of the most commonly involved extrapulmonary sites in sarcoidosis. Hepatic sarcoidosis has a broad range of presentations from scattered, asymptomatic noncaseating granulomas with normal liver enzymes, which are very common in patients with known pulmonary sarcoidosis, to portal hypertension and cirrhosis, which are relatively uncommon. Diagnosis is based on a combination of clinical, laboratory and histological manifestations. The authors’ protocol for management of patients with suspected sarcoidosis of the liver without focal lesions includes a transjugular liver biopsy with portal pressure measurements to confirm the diagnosis, rule out coexisting liver diseases and to identify select patients with fibrosis or portal hypertension for consideration of immunosuppression. Steroids and azathioprine are the preferred agents and methotrexate is not recommended.

Keywords:

• clinical presentation
• hepatic
• management
• review
• sarcoidosis

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• The liver is one of the most commonly involved extrapulmonary sites in sarcoidosis.

• Diagnosis of hepatic sarcoidosis is based on a combination of clinical, laboratory and histological manifestations.

• Hepatic sarcoidosis commonly presents with abnormal elevation of transaminases or with intrahepatic cholestasis but can also present with portal hypertension and cirrhosis.

• Our protocol for the management of patients with suspected sarcoidosis of the liver without focal lesions includes a transjugular liver biopsy with portal pressure measurements to confirm the diagnosis and rule out coexisting liver diseases.

• Steroids and azathioprine are the preferred agents and methotrexate is not recommended.

• Anti-inflammatory agents should be considered for treatment in patients with advanced stages of hepatic sarcoidosis (including liver fibrosis, portal hypertension or worsening of liver function due to active inflammation).

• Liver transplantation should be considered in patients with decompensated cirrhosis and the overall post-transplant outcomes are good.

Notes