Liver Sarcoidosis: Symptoms, Causes, And Treatment

Liver sarcoidosis (hepatic sarcoidosis) is a systemic multiorgan inflammatory disease. Sarcoidosis with some degree of liver involvement comprises approximately 50–65% of all cases.¹

In this article, we cover what liver sarcoidosis is, its symptoms and causes, diagnostic techniques, and potential treatment options. 

What is liver sarcoidosis?

Before we dive into liver sarcoidosis, it’s good to know what sarcoidosis involves in general.

The definition of sarcoidosis is an inflammatory, multisystem disorder involving the formation of small and swollen tissues (granulomas). Sarcoidosis can occur in various organs, with the lymph nodes and lungs being the most common sites. It may also affect the liver, eyes, skin, joints, kidneys, and heart. 

Sarcoidosis is a rare disorder, affecting approximately 2–60 per 100,000 worldwide. It’s more common in northern European countries, Japan, and the US. 

Liver sarcoidosis symptoms 

The impact of sarcoidosis varies depending on the severity and which organs it affects. Whether you have acute or chronic sarcoidosis determines whether symptoms develop suddenly or occur gradually, preventing organs from working properly. 

Symptoms may include:

• Tender bumps on the skin

• Swollen glands in the face, armpits, or groin

• Tiredness and fatigue 

• Painful joints

• Headaches 

In the case of liver involvement, most people will have no symptoms, and about 60–80% will have a normal liver function test. Symptoms may only exist in approximately 5–20% of cases, with the most common ones including:

• Enlarged liver and spleen

• Abdominal pain

• Night sweats

• Weight loss

• Fever

• Joint pain

In rare cases, if the condition progresses, liver damage (cirrhosis) or failure can occur, presenting with the following symptoms: 

• Itchy skin 

• Abdominal pain

• Jaundice (yellow skin and eyes)

• Abdominal edema (ascites)

• Dark urine

How does sarcoidosis affect the liver? 

The disease causes very small epithelial granules in the liver that your doctor can spot easily with a biopsy. Hepatic sarcoidosis doesn’t cause liver cell death. However, it may lead to cholestasis, which is a reduction or blockage in bile flow. 

In rare cases, if the disease advances, it can lead to cirrhosis (liver scarring). This is relatively rare, with an incidence rate of less than 1%.²

Causes of liver sarcoidosis

The formation of hepatic granulomas may be for no apparent reason, as sarcoidosis has an unknown cause. Researchers believe sarcoidosis is multifactorial, involving the input of several genetic, environmental, and immunological factors. These may lead to an autoimmune or immune-mediated response, causing inflammation and granulomas.³

Several risk factors may increase the chance of developing hepatic sarcoidosis, including:

• Exposure to certain infections or chemicals if you’re genetically predisposed

• Being female

• Being 20–40

• Being Black, Japanese, or northern European

• Prior exposure to pegylated interferon (a type of medication)

Diagnosis of liver sarcoidosis

Diagnosing liver sarcoidosis may be difficult. Health professionals need to differentiate it from other diseases, including lymphoma and infections. Because of this, diagnosis usually involves several laboratory and clinical tests. 

Blood tests

In many cases, your liver enzyme test may be normal. However, the most common abnormal blood test result in liver sarcoidosis is elevated alkaline phosphatase. The test may also detect elevated levels of calcium, alanine transaminase (ALT), aspartate aminotransferase (AST), and gamma-glutamyl transferase (GGT). 

Imaging

While imaging is not the most sensitive diagnostic option, it can be useful for detecting extrahepatic sarcoidosis (outside the liver) and portal hypertension. 

Radiological studies

These include magnetic resonance imaging (MRI), ultrasonography, and computed tomography (CT) scans, which may show an enlarged liver. 

Liver biopsy

This is the main definitive diagnostic test for hepatic sarcoidosis.

Complications of liver sarcoidosis

The two major potential complications of long-term liver sarcoidosis include portal hypertension and cirrhosis. The overall mortality rate of systemic sarcoidosis is 1–5%. However, death often occurs due to pulmonary, central nervous system, or cardiac complications rather than liver complications.⁴

Portal hypertension is increased pressure within the portal venous system. This system carries blood from various parts of the digestive system, pancreas, and spleen to the liver. 

Damage or fibrosis of the liver tissue is the primary cause of increased pressure in this system, which should normally be at or below 5 mmHg. A higher pressure gradient can signify portal hypertension, which can be life-threatening, especially when associated with cirrhosis. It can lead to hospitalization and the need for a liver transplant. 

Cirrhosis is scarring of the liver due to long-term liver damage. Liver sarcoidosis can cause cirrhosis, but hepatitis, fatty liver disease, and other conditions can also cause it. Cirrhosis is generally associated with serious symptoms and can cause the liver to stop working properly. 

Currently, there’s no treatment to reverse cirrhosis, but options to manage or slow the progression of the disease exist. 

How is liver sarcoidosis treated?

Before administering treatment, your doctor usually investigates or collaborates with other health professionals to determine whether sarcoidosis is apparent in any other organs, as this will affect management. 

The majority of liver sarcoidosis cases are asymptomatic and may require no treatment. Medication is the usual course of action when you need treatment, with doctors prescribing corticosteroids and ursodeoxycholic acid. 

Other treatments that could suppress your immunity may include:

• Methotrexate

• Chlorambucil

• Glutathione

• Cyclosporine

• Thalidomide

• Azathioprine

In severe cases, you may require a liver transplant, although this is rare. 

The lowdown 

Liver sarcoidosis can present with no obvious symptoms in most people. Other people may experience some symptoms, particularly if the disease advances. Treatment for liver sarcoidosis involves checking other organs for sarcoidosis and tailoring medication to accommodate your specific needs. 

People also ask

Is liver sarcoidosis fatal?

Death caused mainly by liver sarcoidosis is rare, as most deaths of sarcoidosis are due to lung, heart, or central nervous system complications. However, early identification of liver sarcoidosis is critical as the disease is progressive. In rare cases, it can cause end-stage liver disease, which increases mortality.⁵

What’s the last stage of liver sarcoidosis?

If you’re at an advanced stage of liver sarcoidosis, you may develop other conditions. These include:

• Chronic cholestasis, where bile cannot flow to the intestines from the liver

• Portal hypertension, where an increase in blood pressure occurs around the liver 

• Cirrhosis, where advanced and usually permanent scarring of the liver occurs