Non-Alcoholic Fatty Liver Disease (NAFLD)
Fatty liver (steatosis, non-alcoholic fatty liver disease) is a condition where the liver becomes infiltrated with fat. The fat is called steatosis, but when there is inflammation or scarring, it is called steatohepatitis or NASH.
Non-Alcoholic Steatohepatitis (NASH)
Nash, as mentioned above, means there is a presence of hepatic steatosis and inflammation with hepatocyte injury (ballooning) with or without fibrosis. This can progress to fibrosis, cirrhosis, liver failure and liver cancer. Up to 20% of the US population has fatty liver, and perhaps as many as 5% have NASH.
NAFLD tends to develop in people who are overweight and/or have Type 2 diabetes or pre-diabetes (insulin resistance), high cholesterol or high triglycerides. There may also be a genetic component to NAFLD. Occasionally, medications can cause NAFLD.
Most people with NASH are between the ages of 40 and 60, although it can be seen in much younger individuals who have risk factors for NASH. It is more common in women than in men.
NAFLD is often a silent condition, with no symptoms. Blood tests and imaging options, including Fibroscan, can confirm a fatty liver diagnosis.
People with NASH have no symptoms until cirrhosis or liver cancer occurs, which may take many years.
Liver biopsy is sometimes done to confirm NASH diagnosis.
Other than diet and exercise, there are currently no specific treatments available for NASH. Clinical trials of new NASH medications may be the best option for some people.
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HBV is a chronic viral infection that can cause inflammation in the liver, which over time can cause liver damage or liver cancer in some people if left untreated.
HCV is a virus that attacks the liver. If HCV is left untreated, it can result in long-term, progressive liver disease. HCV can eventually prevent the liver from functioning properly.
YOU ARE AT RISK Of HBV IF YOU:
• Were born in the U.S., and not vaccinated as an infant, and have parents born in high-risk countries
• Have traveled to countries where HBV is common
• Were born to a mother who has HBV
• Have had contact with infected body fluids (blood, semen, or vaginal secretions)
• Have had unprotected sex with a person infected with HBV
• Have had a tattoo or body piercing with unsterilized tools
• Have shared unsterilized needles
• Have been diagnosed with Hepatitis C and/or HIV
YOU’RE AT RISK of HCV IF YOU:
• Had a blood transfusion or organ transplant before 1992
• Received a blood-clotting product made before 1987
• Are a health care worker and have been injured by a needle stick
• Received any tattoos or body piercings with non-sterile instruments
• Have been on kidney dialysis for a long time
• Have had sex with a person infected with HCV
• Have HIV
• Have ever been in jail or prison
• Were born to a mother who has HCV
• Have ever injected drugs or shared needles with anyone, even once!
An excellent and safe vaccine is available to prevent HBV. Vaccination against HBV is recommended for everyone, particularly those with liver disease.
In the past 5 years, new medications for HCV have been developed which are highly effective and also well-tolerated with very few or no side effects.
Typical treatment for HCV now involves taking a pill or pills daily for 8 to 12 weeks.
More than 95 percent of patients taking these new medications are completely cured of the disease. Once HCV has been treated, the liver can begin to heal.
If you’ve been diagnosed with HCV, you’re not alone. In the United States, as many as 3 million people have HCV. The good news is that recent scientific advances have made treatments for HCV shorter (as little as 8 weeks) and more effective (over 95% cure rates), with very few, if any, side effects.
Autoimmune liver diseases are chronic, inflammatory diseases that usually progress slowly. The three main categories of autoimmune liver disease are autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC).
Primary biliary cholangitis (PBC) is also a liver disease with no known cause but is believed to be related to other autoimmune disorders. Primary biliary cholangitis begins with inflammation of the small bile ducts. The inflammation blocks the flow of bile out of the liver and liver cells, causing inflammation. As inflammation spreads, scar tissue (fibrosis) develops throughout the liver, replacing more and more liver tissue with fibrosis (scarring), leading to cirrhosis in some cases.
Primary Sclerosing Cholangitis (PSC) is a disease of the large bile ducts in which they become inflamed and heavily scarred. Its cause is unknown and at this time there is no treatment known to stop the disease from progressing.
It is often associated with other autoimmune disorders, such as Sjogren’s syndrome (dry eyes, dry mouth), Raynaud’s phenomenon (impaired circulation in hands and feet in response to cold), hyperthyroidism (Graves’ disease or Hashimoto’s thyroiditis) and rheumatoid arthritis.
Most people with AIH do not experience symptoms until their disease is advanced, but early symptoms may include fatigue. AIH sometimes begins with a “flare” with jaundice, fatigue, itching, fever, and right upper quadrant discomfort
Untreated AIH can lead to progressive scarring of the liver (including cirrhosis).
When diagnosed and treated early, AIH often can be controlled with drugs that suppress the immune system, including steroids and azathioprine.
A liver transplant may be an option if autoimmune hepatitis progresses to advanced liver disease.
Diagnosis of AIH maybe made by blood tests (Anti-nuclear Antibody, Smooth Muscle Antibody), but often a liver biopsy is required.
Cirrhosis is a condition where the liver becomes heavily scarred (or fibrotic); this can occur as a result of alcohol, viral hepatitis, fatty liver disease, or any other type of chronic liver disease.
Many patients with cirrhosis have no associated symptoms. This is why cirrhosis is often diagnosed when the late-stage complications of advanced liver disease develop. Fatigue is common with cirrhosis. When patients begin to develop advanced cirrhosis, they may have evidence of muscle loss, jaundice, edema, or fluid build-up in the abdomen called ascites.
Other complications include portal hypertension, esophageal varices, and hepatic encephalopathy.
Cirrhosis can sometimes be diagnosed by imaging tests (such as a CT scan, ultrasound, or MRI) based on a “nodular” or shrunken appearance to the liver.
Additionally, a fibroscan may provide valuable information about liver disease stage and if cirrhosis is present. If there is fluid in the abdomen (ascites), or an enlarged
spleen, these can also be clues to the presence of cirrhosis. Additionally, cirrhosis can also be diagnosed by liver biopsy, although this is often not necessary.
Cirrhosis may not be reversible, but the progression of liver scarring maybe slowed or halted with appropriate treatment.
Upper endoscopies may be prescribed for screening purposes. The exact frequency will be determined by the gastroenterologist performing endoscopies.
LIVER CANCER SCREENING
How do I get screened for liver cancer?
The most common form of liver cancer in patients with cirrhosis is hepatocellular carcinoma (HCC). This cancer can sometimes be diagnosed in patients without cirrhosis but is most common in patients with cirrhosis and chronic liver disease.
Your risk of developing HCC increases the longer you have cirrhosis. Screening for HCC involves a blood test called an alpha-fetoprotein (AFP), as well as some form of liver imaging (ultrasound, CT or MRI). Surveillance can be done every 6-12 months at a minimum. If a lesion is seen, this may prompt additional testing such as detailed imaging, or a biopsy in some cases.
What vaccines do I need?
• Patients with chronic liver disease should be immunized against hepatitis A virus (HAV) and hepatitis B virus (HBV).
• The inactivated (injected) influenza vaccine is recommended annually.
• The Pneumovax (pneumonia) vaccine is also recommended for adults over age 60.
• Other vaccines should be discussed with your provider in clinic.
Hepatocellular carcinoma (HCC) is the most common type of primary liver cancer (cancer that originates in the liver).
The risk of HCC is higher in people with long-term liver diseases including cirrhosis from any cause, hepatitis B, hepatitis C, or fatty liver disease caused by either alcohol or metabolic factors
Many liver cancer cases are related to hepatitis B virus or hepatitis C virus infections. Most people don’t know they have the virus.
Other behaviors and conditions that increase risk for getting liver cancer are
Excessive alcohol use
Cirrhosis (scarring of the liver, which can also be caused by hepatitis and alcohol use).
Having hemochromatosis, a condition where the body takes up and stores more iron than it needs.
Eating foods that have aflatoxin (a fungus that can grow on foods, such as grains and nuts that have not been stored properly).
The diagnosis of HCC is usually made by imaging tests such as ultrasound, CT, or MRI and tumor marker blood tests (alpha feto-protein). In some cases, a liver biopsy may be done to confirm the diagnosis of HCC.
Treatment will depend on the size and location of the HCC, liver function, and overall health. There are multiple ways of treating liver cancer, sometimes used in combination.
Small tumors in patients whose liver disease is not advanced can sometimes be surgically removed. Surgery to remove the cancer and a margin of healthy tissue that surrounds it may be an option for people with early-stage liver cancers who have normal liver function
Liver transplant surgery
Surgery to remove the entire liver and replace it with a liver from a donor may be an option in otherwise healthy people whose liver cancer hasn’t spread beyond the liver
Destroying cancer cells with heat or cold
Ablation (complete eradication) procedures to kill the cancer cells in the liver using extreme heat or cold may be recommended for people who can’t undergo surgery and have small tumors. These procedures include radiofrequency ablation, cryoablation, and ablation using alcohol injection or microwave
Delivering chemotherapy or radiation directly to cancer cells.
Using a catheter that is passed through your blood vessels and into your liver, doctors can deliver chemotherapy drugs (chemoembolization) or tiny glass spheres containing radiation (radioembolization) directly to the cancer cells to block off the artery feeding the tumor
Both oral and intravenous drugs may help slow the progression of the disease in people with advanced liver cancer, especially in those cases where other therapies are not effective or stop working.
Radiation therapy using energy from X-rays or protons may be recommended to shrink the tumors and slow the growth if surgery or other treatments are ineffective. A specialized type of radiation therapy, called stereotactic body radiotherapy (SBRT), involves focusing many beams of radiation simultaneously at one point in your body with great accuracy
Clinical trials offer an opportunity to try new liver cancer treatments that are in development.
Alcohol-related liver disease (ARLD) is caused by excessive consumption of alcohol and is both common and preventable. ARLD presents in three different stages depending on the severity of liver inflammation. The three stages of ARLD are alcohol-related fatty liver, alcoholic hepatitis, and Cirrhosis.
Alcohol-related Fatty Liver
Also called alcoholic steatosis, is the earliest stage and the most common form of alcohol-related liver disease.
It is characterized by an excessive accumulation of fat inside liver cells, which makes it harder for the liver to function.
Usually there are no symptoms, although the liver can be enlarged, and you may experience upper abdominal discomfort on the right side.
Alcohol related Fatty liver occurs fairly soon in almost all people who drink heavily. The condition will usually go away if you stop drinking before fibrosis (scarring) begins to occur.
Alcoholic hepatitis is acute inflammation, or swelling, of the liver accompanied by the destruction of liver cells. This usually occurs after drinking very large amounts of alcohol continuously over a period of days to weeks.
Up to 35 percent of heavy drinkers develop alcoholic hepatitis, which can be severe and even fatal.
Symptoms may include fever, jaundice, nausea, vomiting, abdominal pain and tenderness.
The disease may occur suddenly – after binge drinking for instance and can quickly lead to life-threatening complications, including severe liver scarring (cirrhosis), the most serious complication of alcohol-related liver disease.
About 20 percent of alcoholics and heavy drinkers develop fatty liver, or steatosis. In many cases there are no clinical symptoms except for an enlarged liver (hepatomegaly). Fatty liver can be reversed if alcohol consumption is stopped or significantly reduced, but the condition can lead to death if alcohol consumption is not reduced or stopped. Some biopsies from people with fatty liver show inflammatory changes, an early sign of more serious liver disease.
-National Institute on Alcohol Abuse and Alcoholism-
For most people, moderate drinking will not lead to alcohol-related liver disease. According to the Dietary Guidelines for Americans, moderate drinking is one drink a day for women and two drinks a day for men.
Each of these alcoholic beverages, in the following amounts, is considered one drink and contains the same amount of alcohol:
• One 12-ounce bottle of beer
• One 4-ounce glass of wine
• One 1-ounce shot of hard liquor
If you have chronic liver disease, even small amounts of alcohol can make your liver disease worse. People with alcohol-related liver disease and those with cirrhosis from any cause should abstain from alcohol completely.
Genetic liver diseases are a group of metabolic and genetic defects that typically cause chronic liver disease. They may be found in close relatives of those who have those diseases.
Hemochromatosis is an iron disorder in which the body stores too much iron. The excess iron, if left untreated, can damage joints and organs (including the liver, pancreas, and heart).
Alpha 1 Antitrypsin Deficiency
Alpha 1 Antitrypsin Deficiency is a rare disease that is caused by the lack of a specific enzyme. This leads to a buildup of abnormal Alpha 1 Antitrypsin in the liver that can eventually cause liver disease as well as lung disease.
Wilson Disease is a genetic disease that prevents the body from removing extra copper. High copper levels can cause life-threatening damage to the body’s organs. Wilson disease causes liver damage, which can be slowly progressive or acute and very severe.
Gilbert syndrome is a mild inherited disorder of the liver that results in an overabundance of a substance known as bilirubin. People with Gilbert syndrome have an inherited abnormality that causes reduced production of an enzyme involved in processing bilirubin. It is not a disease and does not cause illness or limit life expectancy.
Symptoms may include joint pain, fatigue, unexplained weight loss, a darkening of the skin to a bronze color, abdominal pain, loss of sexual function.
In addition to liver disease, people with hemochromatosis may also have diabetes, heart disease and infertility.
Untreated people with hemochromatosis may develop liver cancer and cirrhosis.
Hemochromatosis is diagnosed through both iron and genetic blood tests. In some cases, a liver biopsy is done to assess excess iron in the liver.
The recommended treatment is therapeutic phlebotomy, a procedure that removes blood from the body to reduce iron overload.
Family members of people with hemochromatosis should also be screened.
Diagnosis is made through blood test or mouth swab test.
There is currently no treatment for Alpha 1 Antitrypsin deficiency related liver disease.
Patients who develop cirrhosis and liver failure as a result of Alpha 1 Antitrypsin deficiency can undergo a liver transplant as long as they do not have significant lung disease.
Wilson Disease often does not cause noticeable symptoms until it is serious.
Tests to diagnose include blood and urine tests, eye exam, liver biopsy, and genetic testing.
Treatment is aimed at removing excess accumulated copper and preventing reaccumulation.
With proper therapy and medication, disease progression can be halted and often symptoms can be improved or new symptoms prevented.
While some people with Gilbert syndrome develop yellowing of the skin or eyes (jaundice), most people have no symptoms at all.
Since most patients with Gilbert syndrome have no symptoms. It is often diagnosed incidentally when a lab test done for another reason shows a high level of bilirubin.
Gilbert syndrome is not dangerous and does not cause long-term problems, so it is not necessary to treat Gilbert syndrome.
A liver transplant can help save your life when your liver no longer works or has a disease that can be cured with a liver transplant, such as primary liver cancer. A liver transplant involves surgical removal of your entire liver. It’s then replaced with all, or part, of a healthy donor liver.
If your provider thinks you may be a good candidate for a liver transplant, he or she will refer you to a transplant center for evaluation.
You will have a variety of tests done by the transplant center team. They will decide whether you are a good candidate to receive a transplant. The transplant center team will include:
• A transplant surgeon
• A transplant provider specializing in treating the liver (a hepatologist)
• Transplant nurse coordinators
• A social worker
• A psychiatrist or psychologist
• A nutritionist
• A financial counselor
Psychological and social evaluation
Many different issues are assessed. They include stress, financial concerns, and whether you will have support from family or friends after your surgery.
These tests are done to help find a good donor match and assess your priority on the waiting list.
Tests may be done to check your liver and your general health. These tests may include X-rays, ultrasounds, a liver biopsy, heart and lung tests, colonoscopy, and dental exams. Women may also have a Pap test, gynecology exam, and a mammogram.
You may not be able to have a transplant if you:
• Have a current or chronic infection that can’t be treated
• Have metastatic cancer. This is cancer that has spread from its main location to one or more other parts of the body.
• Have severe heart or lung problems or other health problems
• Have a serious condition besides liver disease that would not get better after a transplant.
• Are not able to follow a treatment plan
• Drink too much alcohol
• Lack of social support or inability to follow the guidance of the transplant program.
You must take medicines for the rest of your life to keep the transplanted liver healthy. These medicines are called anti-rejection medicines (immunosuppressive medicines). They modify your immune system’s response to prevent recognition of the liver as foreign.
In most cases you will take several different anti-rejection medicines at first. The doses may change often, depending on how you respond to them.
Because anti-rejection medicines affect the immune system, people who have a transplant are at a slightly higher risk for certain infections.
The symptoms of rejection may look like other health problems. Talk with your transplant team about any concerns you have. It is important to see them and speak with them often.
Providers at AZ Liver Health are trained and experienced in evaluating and managing liver transplant patients and maintain a referral relationship with liver transplant programs in the region and nationally. Contact our liver clinic to learn more.