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Review
. 2020 Mar 12:12:295-305.
doi: 10.2147/CLEP.S245642. eCollection 2020.

Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review

Kevin Yan  1   2 , Kamal Desai  1 , Lakshmi Gullapalli  1 , Eric Druyts  1 , Chakrapani Balijepalli  1
Affiliations
Review

Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review

Kevin Yan et al. Clin Epidemiol. .

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: aHUS; incidence; kidney; orphan; prevalence; rare.

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Conflict of interest statement

The authors declare that there are no conflicts of interest regarding the publication of this paper.

Figures

Figure 1
Figure 1
PRISMA study flow diagram. Adapted from Moher D, Liberati A, Tetzlaff J, Altman DG, The PRISMA Group (2009) Preferred Reporting Items for Systematic Reviews and Meta-Analyses: The PRISMA Statement. PLoS Med 6(7): e1000097. Creative Commons license and disclaimer available from: http://creativecommons.org/licenses/by/4.0/legalcode.
Figure 2
Figure 2
Studies reporting countries with reported incidence estimates by age group. Note: *Jenssen 2014 reported incidence <1 per million annually (Range: 0–3).
Figure 3
Figure 3
Studies reporting countries with reported prevalence estimates by age group.
See this image and copyright information in PMC

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