Ehlers-Danlos syndrome can raise risk of chronic pancreatitis: Report
Another rare case highlights need for increased awareness, researchers say
Researchers cited a need for increased awareness of Ehlers-Danlos syndrome (EDS) as a risk factor for chronic pancreatitis in a recent case report.
In a rare occurrence, a woman who had chronic pancreatic damage from a previous bout of acute pancreatitis experienced a new symptom attack without an obvious cause, according to the report. Her symptoms of pancreatitis, where the pancreas is progressively inflamed and damaged, were eased with pain medications and avoidance of disease triggers.
Scientists believe the fragile tissues caused by EDS could predispose patients to pancreatitis attacks, even when they don’t have other risk factors, and that doctors and patients alike be made aware of this infrequently described association to avoid more serious complications.
“This case suggests a need for increased awareness of EDS as a risk factor for pancreatitis and emphasizes the importance of lifestyle counseling to prevent triggers,” researchers wrote.
The report, “Acute-on-Chronic Pancreatitis in a Patient With Ehlers-Danlos Syndrome: A Rare Association,” was published in Cureus.
Acute pancreatitis reported in EDS patients
EDS encompasses a group of genetic disorders where the production of important connective tissue proteins is disrupted, causing tissues to become excessively fragile. This affects the skin and joints, as well as the blood vessels or internal organs in some cases.
There have been reports of acute pancreatitis in people with EDS. The condition is characterized by sudden attacks of inflammation in the pancreas, which is responsible for producing digestive enzymes and the blood sugar-regulating hormone insulin. Symptoms include severe abdominal pain, nausea, vomiting, or fever.
With chronic pancreatitis, long-term pancreatic inflammation progressively damages the organ and can influence its normal functions. Before this study, there had only been one other report of chronic pancreatitis in a person with EDS, according to the authors.
The 32-year-old woman in the case report came to the researchers’ clinic with signs of pancreatitis, including left upper abdominal and back pain.
Two years prior, she’d been hospitalized and undergone multiple procedures to treat a complicated case of gallstone pancreatitis, where pancreatitis happens because hardened bile deposits from the gallbladder block the ducts through which pancreatic digestive enzymes usually flow.
Six months after that initial bout of pancreatitis, she had been diagnosed with EDS due to hallmark symptoms of stretchy skin, overly mobile joints, and joint pain.
Chronic pancreatitis likely a result of tissue death from initial bout
At the researchers’ hospital, blood tests showed signs of inflammation or infection. Abdominal imaging also revealed evidence of acute pancreatitis, including swelling and streaks of fat tissue that are indicative of inflammation. There were also signs of chronic pancreatitis, including tissue atrophy and scarring.
The patient was diagnosed with acute-on-chronic pancreatitis, where a person with chronic pancreatitis has a sudden bout of worsening symptoms reminiscent of an acute pancreatitis attack.
While the scientists believe chronic pancreatitis was likely a result of tissue death from the previous bout of pancreatitis, a trigger for this sudden symptom worsening wasn’t identified.
The woman was treated with supportive care including fluids, pain medications, and gradual food introduction.
The woman was ultimately able to eat a low-fat diet and achieved adequate pain control. She was discharged with instructions to avoid pancreatitis triggers by sticking to a low-fat diet and avoiding alcohol. She was also advised to see a pancreatitis specialist.
Follow-up imaging showed pancreatic cysts consistent with chronic pancreatitis, but with adherence to the appropriate diet, the woman reported reductions in symptoms and good pain control.
This supports our hypothesis that some of the [chronic pancreatitis] cases considered idiopathic [without a known cause] at this time may be related to underlying EDS or potentially even other connective tissue diseases.
While her pancreatitis symptoms were similar to what would be expected in the general population, the researchers noted what was particularly striking about this case was the lack of any typical pancreatitis triggers, such as heavy alcohol use, certain medications, elevated fats in the blood, or other inflammatory diseases.
That could mean the underlying EDS itself may have made the woman more susceptible, according to the scientists.
“This supports our hypothesis that some of the [chronic pancreatitis] cases considered idiopathic [without a known cause] at this time may be related to underlying EDS or potentially even other connective tissue diseases,” the researchers wrote.
They believe connective tissue fragility in EDS may cause the body’s small blood vessels to become leaky, leading to an inflammatory environment in the pancreas.
“Further investigation is … required to fully understand any potential connection between EDS and [chronic pancreatitis],” the scientists wrote, adding that studies on the role of genetic factors are “particularly interesting avenues for future studies.”
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