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Multicenter Study
. 2017 Aug 7;12(8):1237-1247.
doi: 10.2215/CJN.00280117. Epub 2017 Jun 8.

Hemolytic Uremic Syndrome in Pregnancy and Postpartum

Alexandra Bruel  1 , 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
Affiliations
Multicenter Study

Hemolytic Uremic Syndrome in Pregnancy and Postpartum

Alexandra Bruel et al. Clin J Am Soc Nephrol. .

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.

Keywords: Antibodies, Monoclonal, Humanized; Atypical Hemolytic Uremic Syndrome; Complement Pathway, Alternative; Female; Follow-Up Studies; France; Humans; Italy; Kidney Failure, Chronic; Plasma Exchange; Postpartum Period; Pregnancy; Recurrence; Retrospective Studies; Thrombotic Microangiopathies; United Kingdom; chemotactic factor inactivator; complement; eculizumab; hemolytic uremic syndrome; kidney transplantation; pregnancy; renal dialysis; thrombotic microangiopathy.

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Figures

Figure 1.
Figure 1.
Pregnancy-associated HUS occured mainly in the postpartum period. HUS, hemolytic uremic syndrome.
See this image and copyright information in PMC

References

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