SARCOIDOSIS OF THE LIVER

Sarcoidosis of the Liver affects the majority of patients with sarcoidosis (up to 70%). However most of these patients rarely or never show symptoms in the liver. They are known as asymptomatic patients. Find out more about liver sarcoidosis below.

The information on this page has been compiled with the help of sarcoidosis specialist Dr Deepak Joshi, Consultant Hepatologist, Kings College Hospital, London.

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Sarcoidosis and the Liver

Symptoms

Symptoms occur in about 20% of patients affected by liver sarcoidosis. These symptoms include:

• Abdominal pain
• Itchy skin
• Fever
• Weight loss
• Hepatomegaly (enlargement of the liver, present in up to 20% of patients)
• Jaundice (yellow skin, present in less than 5% patients)

Diagnosis

Sarcoidosis of the liver will usually be picked up when testing for sarcoidosis in other parts of the body. Symptoms (listed above) will be recognised and investigated further using one or a combination of the tests below:

• Hepatic function test. This shows elevated serum alkaline phosphatase (ALP) and gamma glutamyl transpeotidase (GGT).
• Liver biopsy. This will confirm the presence of granulomas in the liver.
• CT scan. This will show any granulomas (small areas of inflammation) in the liver and can also demonstrate signs of cirrhosis (small, nodular liver).

Rare Conditions

In some rare and chronic cases, liver sarcoidosis can manifest as other conditions. These include:

1. Chronic cholestasis
2. Portal hypertension
3. Cirrhosis

1. Chronic Cholestasis

Patients with advanced hepatic sarcoidosis may develop a chronic cholestatic syndrome. This is where bile cannot flow from the liver to the intestine.

Symptoms:

• Jaundice
• Fever
• Malaise
• Weight Loss
• Anorexia
• Pruritus (itchy skin)

Diagnosis: Cholestatic pattern of abnormal liver function tests.

Treatment: There are limited treatment options. Corticosteroids in doses of 30 to 40 mg/day of prednisone may improve symptoms, lower serum ALP and GGT levels and improve hepatomegaly. Ursodeoxycholic acid may improve liver function tests.

2. Portal hypertension

Portal hypertension is an increase in blood pressure in the veins around the liver. This condition often develops with hepatic sarcoidosis as a result of biliary fibrosis or cirrhosis. It is more likely in advanced patients.

Symptoms:

• Ascites (fluid in the abdomen)
• Bleeding from dilated blood vessels (varices) in the gastro-intestinal (GI) tract

Diagnosis: Abdominal ultrasound and upper GI endoscopy.

Treatment: Diuretics may be given for ascites. Betablockers can help reduce the pressure in the portal venous system. For bleeding varices, therapeutic endoscopy is required.

3. Cirrhosis

Cirrhosis is advanced scarring (fibrosis) of the liver which is usually permanent. This occurs in less than 1% of liver sarcoidosis cases.

Symptoms:

• Fatigue
• Bleeding and bruising easily
• Itchy skin
• Jaundice
• Ascites
• Loss of appetite
• Confusion (hepatic encephalopathy)

Treatment: Standard treatment of cirrhosis and its complications. Please refer to the British Liver Trust. Patients with cirrhosis should be enrolled in a surveillance programme for hepatocellular carcinoma.

Treatment and Outlook

Most patients with liver sarcoidosis have a mild form of the disease and do not require treatment. Up to 75% of patients show improvement without corticosteroids and the rest remain stable.

However treatment with corticosteroids may be necessary for advanced cases. This can improve liver function tests and help alleviate symptoms. The optimal duration of corticosteroids is unclear. All patients with cirrhosis should be referred to a liver specialist or gastroenterologist.

Liver transplantation is a valid option for patients with decompensated liver disease (e.g. development of ascites, Hepatocellular carcinoma (HCC), variceal bleeding).

Page last updated: January 2023. Next review: January 2024.