Scientists urge heart screening for aHUS patients
Study shows pericardial effusion may affect all types of HUS
Pericardial effusion — a potentially life-threatening disorder marked by the buildup of fluid around the heart — can affect people with atypical hemolytic uremic syndrome (aHUS), though it may not cause obvious symptoms, and aHUS patients should be screened for it, scientists said.
The scientists, after reading a report noting that people with typical HUS can experience pericardial effusion, wanted to highlight that the condition can affect those with aHUS as well. They reported on four children with aHUS who visited their clinic and were found to have pericardial effusion. All had antibodies targeting a complement protein called factor H. These antibodies can cause aHUS in some cases.
The study, “Is pericardial effusion restricted to STEC-HUS? Observations in anti-factor H associated atypical hemolytic uremic syndrome,” was published as a letter to the editors in Pediatric Nephrology.
aHUS is a rare disorder marked by the overactivation of part of the immune system called the complement cascade, leading to inflammation and clotting in small blood vessels, which can damage the kidneys and other organs.
Typical HUS is marked by similar symptoms, but with a different underlying cause. Instead of being driven by abnormal complement activity, typical HUS is usually caused by a bacterial infection.
No obvious symptoms
In three of the four cases in the report, mild pericardial effusion seen on imaging scans later resolved.
The fourth case, an eight-year-old girl with a large pericardial effusion that was causing pulse abnormalities and difficulty breathing, was more serious. Because pericardial effusion can be life-threatening when it reaches such an extent, clinicians decided to place a catheter to drain the fluid.
All four children had high blood pressure. The researchers noted, however, that none reported chest pain, and they generally didn’t have alterations in blood parameters that have been documented in people with typical HUS experiencing pericardial effusion. None of them showed obvious signs of heart muscle dysfunction.
The findings show that pericardial effusion can affect people with both typical and atypical HUS, but it may not cause obvious symptoms. This led the researchers to call for efforts to screen for this issue in all HUS patients.
“These observations indicate a need for routine [heart imaging] screening in all HUS cases, irrespective of [underlying cause] or cardiac symptoms,” they wrote.
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