What Is Exocrine Pancreatic Insufficiency? Symptoms, Causes, Diagnosis, Treatment, and Prevention

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Exocrine pancreatic insufficiency, also called EPI, is a condition affecting the pancreas that hinders the body’s ability to digest food, according to the American Gastroenterological Association (AGA).

Typically, the pancreas produces enzymes that break down food and keep digestion moving properly. In people with EPI, the pancreas doesn’t make enough of these enzymes. As a result, digestion is disrupted and the body can’t absorb the nutrients it needs from food.

Signs and Symptoms of Exocrine Pancreatic Insufficiency

EPI can lead to a number of gastrointestinal symptoms, ranging from mild to extremely painful.

According to the AGA, symptoms of EPI can include:

Since people living with EPI aren’t absorbing fats and other important nutrients from food, malnutrition can sometimes occur. This can lead to nondigestive symptoms, including fatigue, dizziness, dry skin, brittle nails, hair loss, depression, irritability, and problems with memory and concentration, according to the Cleveland Clinic.

Learn More About Symptoms of EPI.

Causes and Risk Factors of Exocrine Pancreatic Insufficiency

According to the National Pancreas Association, EPI is often the result of diseases and conditions affecting the pancreas.

These conditions range from genetic conditions that are inherited to ones that develop later in life.

Common risk factors for EPI include:

According to the Cleveland Clinic, EPI may also be caused by celiac disease, diabetes, Crohn's disease, ulcerative colitis, Shwachman-Diamond syndrome (SDS), and surgery of the digestive tract, including weight loss surgery.

Learn More About Causes of EPI.

How Is Exocrine Pancreatic Insufficiency Diagnosed?

If your doctor suspects you may have EPI, there are a number of diagnostic tests available. According to the AGA, the following are common tests used to diagnose the condition:

  • Fecal Elastase Test This is a stool test that detects the enzyme elastase that is released during digestion. Typically, this enzyme is found in stool, so if there is little or none present, it could mean you have EPI.
  • Fecal Fat Test This test looks at the amount of fat in stool. Since fat isn’t being absorbed by the body with EPI, fatty stools may be a sign of the condition.
  • Secretin Pancreatic Function Test This test measures the pancreas’s response to secretin. This hormone is responsible for triggering the release of digestive enzymes from the pancreas to the duodenum, the first part of the small intestine. During the test, secretin is administered into the body through an intravenous, or IV, line. A healthcare provider collects duodenum fluids during an endoscopic ultrasound to be tested for enzymes.

According to the Cleveland Clinic, other tests may include a computerized tomography (CT) scan, abdominal ultrasound, or other imaging tests that would allow a healthcare provider to detect issues with the pancreas that can lead to EPI.

Duration of Exocrine Pancreatic Insufficiency

Because most of the conditions causing EPI, such as pancreatitis and cystic fibrosis, are chronic, EPI itself is also long-lasting. EPI can be managed with medication and most people will need to always continuously be on medication, according to the Cleveland Clinic.

Some individuals with EPI caused by the genetic condition SDS may have a different outcome. According to the Shwachman-Diamond Syndrome Foundation, about half of all patients with SDS show improvement with age and begin producing their own digestive enzymes. In these instances, medication may be stopped under the guidance of a doctor.

Treatment and Medication Options for Exocrine Pancreatic Insufficiency

The main treatment for EPI is pancreatic enzyme replacement therapy, also called PERT. This prescription medication replaces the digestive enzymes lipase, protease, and amylase the pancreas is no longer producing in people with EPI, according to the National Pancreas Foundation.

The U.S. Food and Drug Administration (FDA) has approved six PERT medications, including:

  • Creon, which became the first PERT medication to receive approval in 2009
  • Zenpep
  • Pancreaze
  • Ultresa
  • Viokace
  • Pertzye
According to the AGA, PERT must be taken with food and should be taken at the start of each meal.

The AGA recommends people with EPI eat a healthy diet rich in fruits, vegetables, whole grains, and healthy proteins. It’s also important not to avoid foods with fats, as these are essential to maintaining a healthy weight. However, it’s best to stick with healthy fats, like salmon, avocado, and nuts rather than unhealthy trans or saturated fats found in red meats, dairy products, and fried foods.

Your doctor may also prescribe vitamin and mineral supplements. According to the National Pancreas Foundation these can include vitamins A, D, E, and K.

Learn More About Treatment for EPI.

Prevention of Exocrine Pancreatic Insufficiency

Taking care of your liver is a good way to prevent EPI. This means avoiding smoking and limiting alcohol use, according to the Cleveland Clinic.

Both of these substances cause the pancreas to work on overdrive and can lead to pancreatitis, a common risk factor of EPI. Talk to your doctor if you need help to stop using alcohol or tobacco.
Other causes of EPI are inherited, such as cystic fibrosis and Shwachman-Diamond syndrome, and it’s not possible to prevent EPI with these conditions, the Cleveland Clinic reports.

However, it’s important to tell your doctor about any new symptoms so EPI can be detected and treated right away if it does arise.

Complications of Exocrine Pancreatic Insufficiency

Since people with EPI can’t absorb important nutrients from food, the biggest complication of the condition is malnutrition. According to Johns Hopkins Medicine, symptoms of malnutrition include:

  • Pale, dry skin
  • Changes in skin color
  • Bruising easily
  • Rashes
  • Sensitivity to light
  • Bleeding gums
  • Achy joints
  • Thinning hair or hair that falls out easily
  • Swollen or cracked tongue
EPI can also lead to a number of vitamin deficiencies. According to the Cleveland Clinic, vitamin D deficiency can cause soft, weak bones, which can cause pain and weakness. Too little vitamin D can also result in a condition called osteoporosis, or the loss of bone density.

Vitamin A deficiency is also common in people with EPI. According to the American Academy of Ophthalmology, deficiency in vitamin A can lead to vision problems like night blindness, or difficulty seeing in the dark.

Research and Statistics: How Many People Have Exocrine Pancreatic Insufficiency?

According to a review article published in 2019 in the journal Clinical and Experimental Gastroenterology, the true prevalence of EPI is not known.

However, the paper reports estimates of EPI occurrence in a number of health conditions affecting the pancreas.

For example, about 60 to 90 percent of people with cystic fibrosis will develop EPI within the first 10 to 12 years of diagnosis, the study authors report. People with chronic pancreatitis have a 30 to 90 percent chance of developing EPI, while unresectable pancreatic cancer causes EPI in 20 to 60 percent of patients, according to the report.

Related Conditions and Causes of Exocrine Pancreatic Insufficiency

EPI occurs with conditions affecting the pancreas. Two of the most common causes of EPI include:

Chronic Pancreatitis This condition is characterized by chronic inflammation of the pancreas that does not heal or improve over time. The inflammation can negatively impact the pancreas’s ability to produce digestive enzymes, leading to EPI. According to Mayo Clinic, risk factors for pancreatitis include heavy alcohol use, abdominal surgery, gallstones, an infection, obesity, high calcium or triglyceride levels in the blood, certain medications, cystic fibrosis, and pancreatic cancer.

The National Pancreas Foundation reports about 50 out of every 100,000 people have chronic pancreatitis.

Cystic Fibrosis This is a chronic, progressive genetic disorder that causes severe damage to the lungs and affects the ability to breathe over time, according to the Cystic Fibrosis Foundation.

In people living with cystic fibrosis, sticky, thick mucus builds up in the lungs and other organs, including the pancreas. This prevents them from working properly. According to Mayo Clinic, the sticky mucus can block the digestive enzymes produced in the pancreas from reaching the small intestine, resulting in EPI.

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