Case highlights potential co-occurrence of pancreatitis, aHUS
Clinicians need to 'be vigilant' in assessing for rare disease: Report
Inflammation of the pancreas, known as pancreatitis, can occur alongside the rare disease atypical hemolytic uremic syndrome (aHUS) — as a team of researchers from China highlighted in a new report outlining the case of a young woman found to have both conditions.
The woman sought medical attention due to gastrointestinal issues, including abdominal pain and nausea. Testing showed anemia, or low levels of hemoglobin — the protein that carries oxygen through the bloodstream — and other signs that suggested aHUS, according to the team. The patient also was found to have signs of pancreatitis.
The researchers noted that her case highlights that the two conditions can co-occur, and that clinicians should keep an eye out for this.
“If clinical symptoms … are present, it is important to be vigilant against aHUS,” the researchers wrote. “Although the patient could be diagnosed with acute pancreatitis based on clinical signs, … the etiology [cause] of pancreatitis cannot be determined from various examinations and medical history.”
The study, “Atypical hemolytic uremic syndrome associated with acute pancreatitis,” was published as a letter to the editor in the journal Clinical Nephrology.
Woman, 29, found to have both pancreatitis and aHUS
aHUS is marked by inflammation and clotting in small blood vessels, which can cause damage to internal organs, particularly the kidneys. The disease is often triggered by conditions — such as infections or pregnancy — that alter the activity of the complement system, a part of the immune system. Pancreatitis can also act as a trigger for aHUS, but there have been only a few reported cases of aHUS triggered by pancreatitis.
Here, a quartet of researchers from the First Hospital of Hebei Medical University reported the case of a 29-year-old woman who sought medical attention for abdominal pain, nausea, and vomiting that had lasted for more than a week. The woman also had high blood pressure and an abnormally low urine output, indicative of impaired kidney function.
Laboratory tests revealed anemia and thrombocytopenia, or low numbers of platelets, which are cell fragments that help the blood to clot. Test results also showed elevations in markers of kidney injury and red blood cell destruction.
The combination of anemia due to red blood cell destruction, thrombocytopenia, and kidney injury is a telltale sign of aHUS.
Additional tests ruled out other conditions that can cause similar symptoms, ultimately confirming the diagnosis of aHUS, according to the researchers. Following the diagnosis, the patient was given treatments that included corticosteroids, plasma exchange, and hemodialysis, a procedure to remove waste and extra fluids from the blood. Drug therapy “was too expensive for the patient,” the team noted.
Treatment was successful, per the report, and led to improvements in the woman’s kidney function.
Pancreatitis may be a trigger or a clinical manifestation of [aHUS]. … Early identification of this complication is mandatory for timely treatment and optimal clinical outcomes.
aHUS often develops in people who have underlying mutations in genes that regulate the activity of the complement cascade. Genetic testing did not reveal any such mutations in this patient, however, according to the researchers.
In addition to the aHUS diagnosis, the patient’s clinical workup showed signs of pancreatitis. For example, CT scans of the patient’s abdomen showed signs of fluid accumulation around the pancreas and inflammatory lesions on the organ, which are typical of pancreatitis.
The team stressed that it’s not clear whether pancreatitis triggered this woman’s aHUS, but they said this case highlights that the two conditions can co-occur and that a prompt diagnosis is important.
“Pancreatitis may be a trigger or a clinical manifestation of [aHUS],” the scientists wrote, adding that “early identification of this complication is mandatory for timely treatment and optimal clinical outcomes.”
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