Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

Pediatr Nephrol. 2023 Dec;38(12):4209-4215. doi: 10.1007/s00467-023-06037-2. Epub 2023 Jun 12.

Abstract

Background: Streptococcus pneumoniae-associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS.

Methods: We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center.

Results: The cohort consisted of 4 females and 3 males. All patients had pneumonia. Four were given eculizumab (days 1-3). The eculizumab group required a shorter duration of dialysis and mechanical ventilation (medians 20 vs. 28.5 and 30 vs 38.5 days, respectively) compared with the non-eculizumab group, but this was still much longer than normally reported; the thrombocytopenia resolution was similar in both groups (medians 10 vs. 8 days). Chronic kidney disease (CKD) was correlated with the duration of dialysis and mechanical ventilation duration at 1 year (r = 0.797, P = 0.032 and r = 0.765, P = 0.045) and last follow-up (r = 0.807, P = 0.028 and r = 0.814, P = 0.026, respectively); our scoring system showed even stronger correlations (r = 0.872, P = 0.011 and r = 0.901, P = 0.0057, respectively). The eculizumab group showed slightly better 1-year and last follow-up CKD stage (2.75 vs. 3, P = 0.879 and 2.5 vs. 3.67, P = 0.517).

Conclusions: Despite the fact that the eculizumab group showed better outcomes, eculizumab does not seem to improve the course of P-HUS compared with previous reports. Kidney outcomes are strongly correlated with the duration of dialysis and mechanical ventilation duration. A higher resolution version of the Graphical abstract is available as Supplementary information.

Keywords: Chronic kidney disease; Dialysis; Eculizumab; Hemolytic uremic syndrome; Streptococcus pneumoniae.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Atypical Hemolytic Uremic Syndrome* / complications
  • Atypical Hemolytic Uremic Syndrome* / drug therapy
  • Female
  • Hemolytic-Uremic Syndrome* / complications
  • Hemolytic-Uremic Syndrome* / drug therapy
  • Humans
  • Kidney
  • Male
  • Renal Dialysis
  • Renal Insufficiency, Chronic* / complications
  • Streptococcus pneumoniae

Substances

  • eculizumab