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.
- hemolytic uremic syndrome
- complement
- pregnancy
- thrombotic microangiopathy
- Antibodies, Monoclonal, Humanized
- Atypical Hemolytic Uremic Syndrome
- Complement Pathway, Alternative
- Female
- Follow-Up Studies
- France
- Humans
- Italy
- Kidney Failure, Chronic
- kidney transplantation
- Plasma Exchange
- Postpartum Period
- Pregnancy
- Recurrence
- renal dialysis
- Retrospective Studies
- Thrombotic Microangiopathies
- United Kingdom
- chemotactic factor inactivator
- eculizumab
- Received January 10, 2017.
- Accepted May 5, 2017.
- Copyright © 2017 by the American Society of Nephrology