Rare Classroom: Nonalcoholic Fatty Liver Disease

Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The disease that we will be learning about today is:

Nonalcoholic Fatty Liver Disease

Also called metabolic (dysfunction) associated fatty liver disease.

What is Nonalcoholic Fatty Liver Disease?

  • NAFLD encompasses a histological spectrum from NAFLD, which is characterized by steatosis with no or minor inflammation, to NASH where inflammation and ballooning is present, with or without fibrosis
    • Not considered a rare disease, but diagnosis and management of the condition is lacking​
  • NAFLD is projected to become the leading cause of liver related morbidity and mortality within 20 years and a leading indication for liver transplantation in the next few years
  • Nonalcoholic fatty liver disease (NAFLD) is a condition in which fat builds up in your liver.​
  • Between 30 and 40 percent of adults in the United States have NAFLD. About 3 to 12 percent of adults in the United States have NASH​​
  • Diagnosis of NAFLD includes personal history, blood tests, and imaging tests (ultrasound, CT, MRI). Diagnosis of NASH requires a liver biopsy​
  • Most common form of chronic liver disease in US, affecting an estimated 80 to 100 million people​
  • The prevalence in the morbidly obese population has been estimated as 75-92%, and up to 17% of children and adolescents may be affected with NAFLD

How Do You Get It?

  • Most people with NASH are between the ages of 40 and 60 years​
  • More common in females than in males​
  • NAFLD is more common in people who have certain conditions, including obesity and conditions that may be related to obesity, such as type 2 diabetes. ​
  • Researchers have found NAFLD in 40 to 80 percent of people who have type 2 diabetes and in 30 to 90 percent of people who are obese.​
  • Most common in Hispanics, followed by non-Hispanic whites.​
  • NAFLD is less common in African Americans.​
  • Asian Americans are more likely than people of other racial or ethnic groups to develop NAFLD when their weight is within the normal range.​
  • Other risk factors include:
    • Certain genetic variations
    • Lifestyle factors like habitual snoring
    • Diet
    • Insulin resistance
    • High levels of triglycerides​
    • Abnormal levels of cholesterol—high total cholesterol, high LDL cholesterol, or low HDL cholesterol​
    • Metabolic syndrome or one or more traits of metabolic syndrome. Doctors define metabolic syndrome as the presence of any three of the following:
      • Large waist size​
      • High levels of triglycerides in your blood​
      • Low levels of HDL cholesterol in your blood​
      • High blood pressure​
      • Higher than normal blood glucose levels​
  • Some less common factors include:
    • Disorders that cause your body to use or store fat improperly​
    • Rapid weight loss​
    • Certain infections, such as hepatitis C
    • Exposure to certain toxins​
    • Certain medicines, such as:​
      • Amiodarone (Cordarone, Pacerone)​
      • Diltiazem ​
      • Glucocorticoids ​
      • Highly active antiretroviral therapy ​
      • Methotrexate (Rheumatrex, Trexall)​
      • Synthetic estrogens ​
      • Tamoxifen (Nolvadex, Soltamox)​
      • Valproic acid​

What Are The Symptoms?

  • Physical Symptoms​
    • Often asymptomatic​
    • Fatigue​
    • Weakness​
    • Weight loss​
    • Loss of appetite​
    • Nausea​
    • Abdominal pain​
    • Spider-like blood vessels​
    • Yellowing of the skin and eyes (jaundice)​
    • Itching​
    • Fluid build-up and swelling of the legs (edema) and abdomen (ascites)​
  • Mental/Emotional Symptoms​
    • Mental confusion​

How Is It Treated?

  • The goal of treatment is to reduce liver cell injury and inflammation reduce the risk of cirrhosis, liver failure, and hepatocellular carcinoma​
  • There are no currently approved marketed drugs to treat NASH​
  • Barriers to treatment include difficulty in enacting lifestyle changes such as diet and exercise regimes, lack of pharmacological treatments, and delayed diagnosis​
  • The Health Care Team consists of hepatologists and gastroenterologists as well as endocrinologists who manage diabetes, impaired glucose tolerance, and obesity​
  • Factors impacting treatment/prognosis include speed to diagnosis and ability to implement lifestyle changes​
  • The risk of progression to end-stage liver disease is influenced by the severity of underlying liver histology​
  • Progression to cirrhosis in NASH patients varies with 10% developing decompensated liver disease over 13 years and 25% developing cirrhosis over nine years ​
  • The rate of progression is heavily influenced by the underlying fibrosis stage, with NASH patients without fibrosis at significantly lower risk compared to those with advanced fibrosis ​
  • Progression to advanced fibrosis and cirrhosis is not uniform in all patients, and metabolic factors such as the presence of glucose intolerance and type 2 diabetes mellitus may play a key role in this progression ​
  • Once cirrhosis has developed, the risk of developing a major complication of portal hypertension is 17%, 23%, and 52% at one, three, and 10 years, respectively
    • If NASH leads to cirrhosis, doctors can treat the health problems caused by cirrhosis with medicines, operations, and other medical procedures. If cirrhosis leads to liver failure, you may need a liver transplant.​
  • The survival of patients with NASH cirrhosis falls markedly once decompensation occurs, with a median survival of approximately two years
  • Doctors recommend weight loss to treat nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH). Weight loss can reduce fat in the liver, inflammation, and fibrosis. No medicines have been approved to treat NAFLD and NASH.​

Where Can I Learn More???

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