Decreased Neutrophil Extracellular Trap Degradation in Shiga Toxin-Associated Haemolytic Uraemic Syndrome

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title = "Decreased Neutrophil Extracellular Trap Degradation in Shiga Toxin-Associated Haemolytic Uraemic Syndrome",
abstract = "Background: Neutrophil extracellular traps (NETs) can stimulate thrombosis, and their degradation is decreased in several autoimmune disorders. It was recently reported that some patients with haemolytic uraemic syndrome (HUS) also fail to degrade NETs and that neutrophils from Shiga toxin-associated HUS are primed to form NETs. Method: We used a well-characterized cohort of 74 thrombotic microangiopathy (TMA) patients, with a subset also providing follow-up samples, and 112 age-matched controls to investigate NET degradation and serum nuclease activity in TMA before, during and after treatment. Results: We identified that in the cohort of TMA patients, 50{\%} of patients with Shiga toxin-associated HUS displayed a decreased ability to degrade NETs. NET degradation correlated with serum nuclease activity, but not with autoantibodies against double-stranded DNA, which has been previously observed in some autoimmune disorders. Further, NET degradation negatively correlated with serum creatinine levels, suggesting that kidney function was negatively impacted by the low NET degradation ability. Conclusions: We revealed that decreased NET degradation is a common feature of Shiga toxin-associated HUS and that it is associated with decreased kidney function in these patients. It remains to be clarified whether improving NET degradation would be beneficial for the patient.",
keywords = "Degradation, Kidney injury, Neutrophil extracellular traps, Nuclease activity, Shiga toxin-associated haemolytic uraemic syndrome, Thrombotic microangiopathies",
author = "Jonatan Leffler and Zolt{\'a}n Proh{\'a}szka and B{\'a}lint Mikes and Gy{\"o}rgy Sinkovits and Katarzyna Ciacma and P{\'e}ter Farkas and Marienn R{\'e}ti and Kata Kelen and Reusz, {Gy{\"o}rgy S.} and Szab{\'o}, {Attila J.} and Myriam Martin and Blom, {Anna M.}",
year = "2017",
doi = "10.1159/000450609",
language = "English",
volume = "9",
pages = "12--21",
journal = "Journal of Innate Immunity",
issn = "1662-811X",
publisher = "Karger",
number = "1",