Journal article
A Case-Based Narrative Review of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome/Complement-Mediated Thrombotic Microangiopathy
Kidney international, Vol.105(5), pp.960-970
05/2024
DOI: 10.1016/j.kint.2023.12.021
PMCID: PMC12362266
PMID: 38408703
Abstract
Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated thrombotic microangiopathy (TMA), caused by uncontrolled activation of the alternative complement pathway in the setting of autoantibodies to or rare pathogenic genetic variants in complement proteins. Pregnancy may serve as a trigger and unmask aHUS/complement-mediated TMA [aHUS/CM-TMA] which has severe, life-threatening consequences. It can be difficult to diagnose aHUS/CM-TMA in pregnancy due to overlapping clinical features with other TMA syndromes including hypertensive disorders of pregnancy. However, the distinction among TMA etiologies in pregnancy is important as each syndrome has specific disease management and treatment. In this narrative review, we discuss two cases to illustrate the diagnostic challenges and evolving approach in the management of pregnancy-associated aHUS/CM-TMA. The first case involves a 30-year-old female presenting in the first trimester who was diagnosed with aHUS/CM-TMA and treated with eculizumab from 19 weeks gestation. Genetic testing revealed a likely pathogenic variant in CFI. She successfully delivered a healthy infant at 30 weeks gestation. In the second case, a 22-year-old female developed severe postpartum HELLP syndrome and required hemodialysis. Her condition improved with supportive management, yet investigations assessing for aHUS/CM-TMA remained abnormal 6 months postpartum consistent with persistent complement activation but negative genetic testing. Through detailed case discussion describing tests assessing for placental health, fetal anatomy, complement activation, autoantibodies to complement regulatory proteins, and genetic testing for aHUS/CM-TMA, we describe how these results aided in the clinical diagnosis of pregnancy-associated aHUS/CM-TMA and assisted in guiding patient management including the use of anti-complement therapy.
Details
- Title: Subtitle
- A Case-Based Narrative Review of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome/Complement-Mediated Thrombotic Microangiopathy
- Creators
- Michael Che - University of OttawaSarah M Moran - Cork University HospitalRichard J Smith - University of IowaKevin Y M Ren - Queen's UniversityGraeme N Smith - Queen's UniversityM Khaled Shamseddin - Queen's UniversityCarmen Avila-Casado - University of TorontoJocelyn S Garland - Queen's University
- Resource Type
- Journal article
- Publication Details
- Kidney international, Vol.105(5), pp.960-970
- DOI
- 10.1016/j.kint.2023.12.021
- PMID
- 38408703
- PMCID
- PMC12362266
- NLM abbreviation
- Kidney Int
- ISSN
- 0085-2538
- eISSN
- 1523-1755
- Publisher
- ELSEVIER SCIENCE INC
- Grant note
- DOI: 10.13039/100004325, name: AstraZeneca; DOI: 10.13039/100004330, name: GlaxoSmithKline; DOI: 10.13039/100000062, name: National Institute of Diabetes and Digestive and Kidney Diseases, award: R01 DK110023
- Language
- English
- Electronic publication date
- 02/24/2024
- Date published
- 05/2024
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984563451602771
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