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Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review

Authors Yan K, Desai K, Gullapalli L, Druyts E, Balijepalli C

Received 11 January 2020

Accepted for publication 26 February 2020

Published 12 March 2020 Volume 2020:12 Pages 295—305


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Dr Vera Ehrenstein

Kevin Yan,1,2 Kamal Desai,1 Lakshmi Gullapalli,1 Eric Druyts,1 Chakrapani Balijepalli1

1Pharmalytics Group, Vancouver, British Columbia, Canada; 2Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada

Correspondence: Chakrapani Balijepalli 637 East 15th Ave, Vancouver, British Columbia V5T 3K5, Canada
Tel +1 604-398-4025

Abstract: Atypical hemolytic uremic syndrome (aHUS) is a rare but severe disorder that frequently has a genetic component and results from the overactivation of the alternative complement pathway. As research moves toward improved diagnosis and therapy of aHUS, it will be important to better understand its epidemiology. Our objective was to conduct a systematic literature review to assess the incidence and prevalence estimates of aHUS globally. A comprehensive literature search was conducted in Embase and MEDLINE. Additionally, practice guidelines, databases of national/international organizations, and regulatory agencies were searched. From 2960 publications identified via MEDLINE and Embase, 105 publications were eligible for full-text screening, and a total of eight full-text articles met eligibility criteria for inclusion. Regional epidemiologic estimates were obtained for Europe and Oceania. Country-specific data were available for France, Norway, Australia, and Italy. Four of the identified studies reported on the prevalence of aHUS, prevalence in the age group of 20 years or younger was ranging from 2.2 to 9.4 per million population, while the only study that reported prevalence in all ages showed a prevalence of 4.9 per million population. Six studies reported on the incidence of aHUS, annual incidence in the age group of 20 years or younger was ranging from 0.26 to 0.75 per million population, and for all ages, annual incidence was ranging from 0.23 to 1.9 per million population. To our knowledge, this is the first systematic review conducted to provide a comprehensive overview of global incidence and prevalence estimates of aHUS. In general, incidence estimates were similar across all the studies; however, prevalence data were found to be more variable. Study limitations were related to inconsistencies in the definitions of aHUS between studies and also a dearth of epidemiological studies assessing incidence and prevalence of aHUS outside of Europe.

Keywords: incidence, prevalence, aHUS, rare, orphan, kidney

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