Atypical Hemolytic Uremic Syndrome Associated With Clostridium Difficile Infection

Abstract

Atypical hemolytic uremic syndrome (aHUS), defines as non-Shiga toxin HUS, is thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and renal impairment. aHUS is associated with high morbidity and mortality, necessitating the need for an early diagnosis to limit target organ damage. Mutations or autoantibodies against specific complement factors over-activate the complement system forming microthrombi. aHUS has the potential to cause multi-organ system dysfunction, but it predominantly affects the kidneys. aHUS is treated with eculizumab, a terminal blocker of the complement system. Clostridium difficile infection is a rare precipitant of aHUS. We present a case of aHUS associated with Clostridium difficile infection in a 60-year-old female patient that was successfully treated with eculizumab.

Clostridioides difficile-Associated Atypical Hemolytic-Uremic Syndrome Successfully Treated With Eculizumab: A Case Report and Literature Review

“…Finally, our patient had significant neurologic symptoms to include seizures, EEG, and MRI findings as discussed above, leading to mechanical intubation for several days. In previously reported cases, only four other patients had neurologic symptoms, however none as profound as our patients (2–7). This case highlights that the high morbidity and mortality associated with aHUS likely also present with CD-aHUS and that timely recognition and initiation of appropriate therapy are integral to improving outcomes.…”

“…Primary aHUS has been attributed to infections such as Streptococcus pneumoniae, but a possible relationship with Clostridioides difficile infections has also been described. Our literature review demonstrates nine adult patients diagnosed with C. difficile -associated aHUS (CD-aHUS) (2–7), although the exact mechanism is poorly understood (8). Of these cases, only two have been treated effectively with plasmapheresis and eculizumab.…”

Atypical Hemolytic Uremic Syndrome in the Setting of Acute Clostridium difficile Colitis

“…HUS is a form of thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure [ 6 ]. It was formerly classified into typical HUS, which is caused by Shiga toxin-producing E. coli and considered the most common type in children, and atypical HUS, or non-Shiga toxin HUS, which accounts for most of the adult cases [ 7 ]. The new classification was developed based on the pathophysiology and precipitating factors [ 8 ]; hereditary causes include complement gene mutations and cobalamin C deficiency.…”