What Is Exocrine Pancreatic Insufficiency? Symptoms, Causes, Diagnosis, Treatment, and Prevention
Updated on January 28, 2024
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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 guidelines published by 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:
- Gas
- Bloating
- Diarrhea
- Abdominal pain
- Greasy, oily stools
- Foul-smelling stools
- Unexplained weight loss
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.
Causes and Risk Factors of Exocrine Pancreatic Insufficiency
According to the National Pancreas Foundation (NPF), the cause of EPI is often the result of other 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:
- Chronic pancreatitis
- Cystic fibrosis
- Pancreatic cancer
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.
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 (IV) line. A healthcare provider collects intestinal fluid during an upper endoscopy to be tested for the concentration of pancreatic secretions.
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.
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, which the pancreas inadequately produces in people with EPI, according to the Gastrointestinal Society.
The U.S. Food and Drug Administration (FDA) has approved six PERT medications, which utilize the generic medication pancrelipase in different ways. The brand name medications are:
- Creon
- Zenpep
- Pancreaze
- Ultresa
- Viokace
- Pertzye
According to the AGA, PERT must be taken with food and 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 and monitor vitamin and mineral supplements. According to the NPF, these can include vitamins A, D, E, and K.
Prevention of Exocrine Pancreatic Insufficiency
Avoiding smoking and limiting alcohol use are good ways to prevent EPI, according to the Cleveland Clinic. Both of these substances are toxic to the pancreas 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. 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, a 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, the true prevalence of EPI is not known. However, the review reported 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 reported. 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 review.
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. Research published in 2023 estimated that about 45 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, which 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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Resources
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- Exocrine Pancreatic Insufficiency (EPI). Cleveland Clinic. June 10, 2021.
- Exocrine Pancreatic Insufficiency (EPI). National Pancreas Foundation.
- About SDS. Shwachman-Diamond Syndrome Foundation.
- Pancreatic Exocrine Insufficiency. Gastrointestinal Society.
- Pancreatic Enzyme Replacement Therapy (PERT). Mission:Cure.
- Malnutrition. Johns Hopkins Medicine.
- Vitamin D Deficiency. Cleveland Clinic. August 2, 2022.
- Boyd C. What Is Vitamin A Deficiency? American Academy of Ophthalmology. May 2, 2023.
- Capurso G et al. Exocrine Pancreatic Insufficiency: Prevalence, Diagnosis, and Management. Clinical and Experimental Gastroenterology. March 21, 2019.
- Pancreatitis: Symptoms and Causes. Mayo Clinic. September 23, 2023.
- Cai QY et al. Incidence, Prevalence, and Comorbidities of Chronic Pancreatitis: A 7-Year Population-Based Study. World Journal of Gastroenterology. August 14, 2023.
- What Is Cystic Fibrosis? Cystic Fibrosis Foundation.
- Cystic Fibrosis: Symptoms and Causes. Mayo Clinic. November 23, 2021.