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Hemolytic Uremic Syndrome in Pregnancy and Post-Partum

Lookup NU author(s): Professor David KavanaghORCiD, Dr Edwin Wong, Dr Vicky Brocklebank

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This is the authors' accepted manuscript of an article that has been published in its final definitive form by American Society of Nephrology, 2017.

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Abstract

Background: Pregnancy is associated with various forms of thrombotic microangiopathy, including the hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical HUS linked to complement alternative pathway dysregulation. Design, setting, participants and measurements: We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway genes 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 post-partum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions and 4 (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached end-stage renal disease, 15 (19%) had chronic kidney disease and 18 (28%) patients experienced hemolytic uremic syndrome relapse. Twenty four patients (27%) received a renal transplant and a recurrence of hemolytic uremic syndrome occurred in 13 (54%) cases. 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 non-related to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.


Publication metadata

Author(s): Bruel A, Kavanagh D, Noris M, Delmas Y, Wong E, Bresin E, Provôt F, Brocklebank V, Mele C, Remuzzi G, Loirat C, Frémeaux-Bacchi V, Fakhouri F

Publication type: Article

Publication status: Published

Journal: Clinical Journal of the American Society of Nephrology

Year: 2017

Volume: 12

Issue: 8

Pages: 1237-1247

Print publication date: 07/08/2017

Online publication date: 08/06/2017

Acceptance date: 17/05/2017

Date deposited: 09/08/2017

ISSN (print): 1555-9041

ISSN (electronic): 1555-905X

Publisher: American Society of Nephrology

URL: https://doi.org/10.2215/CJN.00280117

DOI: 10.2215/CJN.00280117


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Funding

Funder referenceFunder name
E.K.S.W. is a Medical Research Council clinical training fellow
D.K. is a Wellcome Trust intermediate clinical fellow
European Union's Seventh Framework Programme (FP7/2007-2013) under Grant 305608 (EURenOmics)
VB is funded by NCKRF

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