Escherichia coli-associated hemolytic uremic syndrome and severe chronic hepatocellular cholestasis: complication or side effect of eculizumab?
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Liver lesions of hemolytic uremic syndrome due to Shiga-toxin-producing Escherichia coli (STEC-HUS) are uncommon.
We report three observations of severe STEC-HUS with delayed hepatic involvement. They presented with multiple organ failure and received eculizumab; 15 days after the onset of STEC-HUS, cholestasis appeared and cytolysis worsened. Abdominal ultrasonography showed vesicular sludge. Liver biopsy performed 3 to 6 months after the STEC-HUS found cholangiolar proliferation and inflammatory portal fibrosis. Despite renal recovery, cholestasis persisted and worsened in two cases, leading to biliary cirrhosis and subsequent liver transplantation. Pathological examination of one native liver found thrombotic microangiopathy.
Even though the pathological examination performed on one native liver demonstrated areas of thrombotic microangiopathy, we cannot completely rule out that eculizumab may have worsened the liver lesions. Before the efficacy of eculizumab in STEC-HUS is formally demonstrated, physicians should stay cautious in its use.
KeywordsHUS Pediatrics Eculizumab Cholestatis Liver transplantation
The authors would like to thank Pr Chantal Loirat for her expertise and skillful help during the management of the cases, as well as Dr Véronique Frémeaux-Bacchi for genetic explorations.
Compliance with ethical standards
This retrospective series of cases was approved by the local IRB (Comité d’éthique des Hospices Civils de Lyon, session May 3, 2017).
Conflict of interest
Justine Bacchetta and Anne-Laure Sellier-Leclerc received travel grants and honoraria from Alexion (2013, 2014).
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