Case of man, 36, highlights link between pancreatitis and aHUS
Researchers: Disease may be triggered by acute inflammation of pancreas
Atypical hemolytic uremic syndrome (aHUS) may be triggered by acute inflammation of the pancreas, or pancreatitis, a team of researchers from India highlighted in a report describing the case of a 36-year-old man with both conditions.
The man sought medical attention due to abdominal pain and was found to have signs of pancreatitis. He later developed anemia, or low levels of hemoglobin — the oxygen-carrying protein in red blood cells — and other signs of aHUS, according to the team.
“This case highlights atypical Hemolytic Uremic Syndrome as a rare complication of Acute Pancreatitis. Our patient, despite being diagnosed well in time and managed aggressively, had an unfavourable outcome. This condition should be diagnosed well in time, as it is associated with high mortality,” they wrote.
The case study, “Hemolytic uremic syndrome in severe acute pancreatitis,” was published in Oxford Medical Case Reports.
Patient sought treatment for abdominal pain
A rare disease, aHUS is caused by the abnormal activity of the complement cascade, a part of the immune system. This leads to increased inflammation and the formation of blood clots inside small blood vessels. These clots can damage internal organs, especially the kidneys.
Although most people with aHUS have genetic mutations that predispose them to develop the disease, usually a triggering event, such as pregnancy or another illness, is also necessary.
Here, the researchers reported the case of a 36-year-old man who developed aHUS secondary to acute pancreatitis.
He initially sought medical attention for abdominal pain that radiated to his back. He reported having consumed alcohol two days before the onset of his symptoms and had a previous history of pulmonary tuberculosis, a bacterial infection affecting the lungs.
Upon physical examination, he had normal heart and breathing rates, and felt pain when pressure was applied to the left side of his abdomen.
Laboratory tests revealed elevated levels of amylase and lipase, two enzymes produced by the pancreas to help digest carbs and fats, which is indicative of pancreatic injury. An abdominal ultrasound suggested the presence of acute pancreatitis, which was confirmed in a CT scan three days after admission.
On the fifth day of admission, he developed a fever, had a fast heart rate, anemia, and a low number of platelets, cell fragments that help the blood to clot. He also had an elevated number of neutrophils, a type of immune cell.
Soliris recommended as treatment for aHUS in acute pancreatitis
Further tests revealed the presence of fragmented red blood cells and increased levels of lactate dehydrogenase, consistent with hemolysis, or red blood cell destruction. He also had high levels of procalcitonin, an indicator of severe infection, and was showing signs of kidney and liver dysfunction.
Doctors suspected bacterial hemolytic uremic syndrome, and the patient began treatment with plasma exchange, antibiotics, and a blood transfusion.
Further analyses revealed his condition was being mediated by a dysfunction of the complement system, leading to the diagnosis of aHUS. He was then treated with plasma exchange, intravenous immunoglobulin (IVIG) therapy, and hemodialysis.
He did not respond to plasma exchange or IVIG, and was started on the immunosuppressant rituximab (sold as Rituxan, among others) 15 days after being admitted to the hospital. Despite intensive treatment, his kidney function continued to deteriorate, and he developed breathing issues before dying 21 days after admission.
We suggest long term [Soliris] with apt supportive care as the mainstay of treatment for aHUS in [acute pancreatitis].
According to the researchers, if the patient had been treated with Soliris (eculizumab), which was not available at their center, the outcome could have been favorable.
Based on literature, 38 cases of aHUS occurring in people with acute pancreatitis of different origins have been reported. Overall, considering the interval between pancreatitis and aHUS onset, it suggests aHUS may be secondary to acute pancreatitis, in the absence of other potential triggers.
“We suggest long term [Soliris] with apt supportive care as the mainstay of treatment for aHUS in [acute pancreatitis],” the researchers wrote. As a second-line alternative, plasma exchange, IVIG, and corticosteroids may be used, as well as rituximab in cases of relapsing or treatment-resistant aHUS.
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