Woman with Cushing’s seen with rare digestive tract perforation

High cortisol, plus bacteria may have led to the condition, researchers said

Steve Bryson, PhD avatar

by Steve Bryson, PhD |

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A woman with Cushing’s syndrome developed an ulcer that caused a gastrointestinal perforation, or a hole in the digestive tract, according to a case study.

Because she had no established risk factors for such a complication, researchers suggested prolonged exposure to high levels of cortisol, or hypercortisolism, may have contributed to it. Hypercortisolism is the underlying cause of Cushing’s.

The findings “should elicit a high clinical suspicion and demand prompt investigation of Cushing’s syndrome patients in a hypercortisolism state presenting with modest gastrointestinal symptoms,” the researchers wrote in “Adrenocorticotropic Hormone-Dependent Cushing’s Syndrome Complicated With Gastric Ulcer Perforation in a 30-Year-Old Saudi Female: A Case Report and a Review of the Literature,” which was published in Cureus.

Cushing’s syndrome encompasses a group of diseases marked by elevated levels of the stress hormone cortisol. Hypercortisolism can lead to weight gain, muscle weakness, skin changes, and extreme fatigue. Cushing’s disease is a subtype of the syndrome and is marked by excess levels of adrenocorticotropic hormone (ACTH) due to tumors in the pituitary gland. In ectopic Cushing’s, another subtype of the syndrome, excess ACTH comes from tumors outside the pituitary gland.

Regardless of the location of a tumor, excess ACTH stimulates the adrenal glands atop the kidneys to produce and release too much cortisol into the bloodstream, leading to Cushing’s symptoms. These two subtypes are considered endogenous forms of Cushing’s syndrome because they’re caused by problems inside the body.

Exogenous Cushing’s syndrome is a form of Cushing’s wherein hypercortisolism is caused by the ongoing use of anti-inflammatory glucocorticoids, which act like cortisol in the body. A well-established complication of this type of hypercortisolism is gastrointestinal perforation — holes that form in the stomach or small intestine, allowing food or digestive fluids to leak into the abdomen. There is limited information on gastrointestinal complications associated with hypercortisolism caused by endogenous forms of Cushing’s syndrome.

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A diagnosis of ACTH-dependent Cushing’s

In this study, researchers in Saudi Arabia described the case of a 30-year-old woman with intellectual disabilities diagnosed with ACTH-dependent Cushing’s who developed a gastrointestinal perforation soon afterward.

She was admitted to the emergency department with progressive weakness and fatigue. An assessment indicated progressive weight gain, menstrual irregularities, and generalized weakness, which limited her physical activity for the past three months.

A physical exam showed signs consistent with hypercortisolism, including abdominal stretch marks, a rounded face, a fat pad between her shoulders, and acne on her face, chest, shoulders, and back. Blood tests revealed excess cortisol, ACTH, and blood sugar levels, along with low levels of potassium (hypokalemia). CT scans of her neck, chest, abdomen, and pelvis failed to detect ectopic tumors, however, and a brain MRI found no signs of tumors in the pituitary gland.

Nevertheless, the findings “supported the diagnostic hypothesis of ACTH-dependent Cushing’s syndrome,” the researchers wrote.

Gastrointestinal perforation confirmed

The woman was treated for low potassium and high blood sugar, but sought emergency care seven days later due to abdominal pain, constipation, and low urine output. An exam showed a swollen abdomen, but it wasn’t painful. Blood tests of kidney function and other parameters were normal. She was treated for abdominal pain and constipation and a follow-up appointment was scheduled.

She returned to the hospital five days later with the same symptoms, but a physical exam revealed swelling in her lower limbs and abdominal muscle tightness. A chest X-ray showed a large amount of air or gas in the abdominal cavity.

Abdominal CT scans confirmed a gastrointestinal perforation, with a hole of about 0.8 cm (0.3 inches), likely related to an underlying ulcer.

An emergency surgery corrected the perforation and a tissue exam confirmed a stomach ulcer with no signs of cancer. She tested positive for Helicobacter pylori, a bacteria that can cause infections in the stomach or small intestine and is the most common cause of peptic ulcers. According to researchers, “the prevalence of Helicobacter pylori among Saudi patients is high.”

After her recovery, the woman was prescribed metyrapone to lower her cortisol levels.

Because she had no established risk factors for gastric perforation, the researchers suggested “prolonged excess glucocorticoids from Cushing’s syndrome may have contributed to the gastric perforation either independently or synergistically with Helicobacter pylori.” They also noted a “perforation may not be contained or healed initially due to the immunosuppressive effects of hypercortisolism.”

“This case report sheds light on one of the less common complications of hypercortisolism in patients with Cushing’s syndrome, which is gastrointestinal perforation,” the researchers wrote.