Hemolytic Uremic Syndrome in Pregnancy and Postpartum

Alexandra Bruel, David Kavanagh, Marina Noris, Yahsou Delmas, Edwin K S Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated hemolytic uremic syndrome.

RESULTS: Eighty-seven patients with pregnancy-associated hemolytic uremic syndrome were included. Hemolytic uremic syndrome occurred mainly during the first pregnancy (58%) and in the postpartum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions, and four (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached ESRD, 15 (19%) had CKD, and 18 (28%) patients experienced hemolytic uremic syndrome relapse. Twenty-four patients (27%) received a kidney transplant and a recurrence of hemolytic uremic syndrome occurred in 13 (54%) patients. Variants in complement genes were detected in 49 (56%) patients, mainly in the CFH (30%) and CFI genes (9%).

CONCLUSIONS: Pregnancy-associated hemolytic uremic syndrome and atypical hemolytic uremic syndrome nonrelated to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.

Original languageEnglish
Pages (from-to)1237-1247
Number of pages11
JournalClinical journal of the American Society of Nephrology : CJASN
Volume12
Issue number8
DOIs
Publication statusPublished - Aug 7 2017

Keywords

  • Journal Article

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    Bruel, A., Kavanagh, D., Noris, M., Delmas, Y., Wong, E. K. S., Bresin, E., Provôt, F., Brocklebank, V., Mele, C., Remuzzi, G., Loirat, C., Frémeaux-Bacchi, V., & Fakhouri, F. (2017). Hemolytic Uremic Syndrome in Pregnancy and Postpartum. Clinical journal of the American Society of Nephrology : CJASN, 12(8), 1237-1247. https://doi.org/10.2215/CJN.00280117