Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis

Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis Publisher Pubmed

Fekrvand S ; Esfahani ZH ; Yarahmadi M ; Saeediboroujeni A ; Salehi H ; Hakimelahi A ; Alamsihashiani A ; Rahmati M ; Afsharqasemloo S ; Namehgoshayfard NNG ; Monfared FT ; Afkham EK ; Fathi N ; Shad TM Show All Authors

Fekrvand S
Esfahani ZH
Yarahmadi M
Saeediboroujeni A
Salehi H
Hakimelahi A
Alamsihashiani A
Rahmati M
Afsharqasemloo S
Namehgoshayfard NNG
Monfared FT
Afkham EK
Fathi N
Shad TM
Babaha F
Nazari F
Nirouei M
Farid AS
Sanadgol N
Rafiemanesh H
Marzbali MY
Hassanpour G
Olbrich P
Condinoneto A
Morio T
Gennery AR
Meyts I
Ochs HD
Abolhassani H
Rezaei N
Yazdani R

Source: Mutation Research - Reviews in Mutation Research Published:2025

Abstract

Background: Patients with inborn errors of immunity (IEI) experience severe infectious and non-infectious complications, leading to an increased risk of mortality. Delayed diagnosis or misdiagnosis significantly contributes to the heightened mortality rates observed in IEI patients. Objectives: This study systematically reviews the causes of mortality in IEI patients with a meta-analysis to determine the mortality rate among patients with various IEI. Methods: Embase, ISI Web of Science, PubMed, and Scopus were searched (up to July 2024) using terms related to IEI and mortality. Results: A total of 12,581 deceased IEI patients were included, with an overall reported mortality rate of 24.0 % (95 % confidence interval: 23.0–26.0 %) among all published IEI cases. This represents an approximately 27-fold higher mortality rate among IEI patients compared to the mean global mortality rate (24 % vs. 0.874 %). Severe combined immunodeficiency, chronic granulomatous disease, and ataxia-telangiectasia had the highest numbers of reported deceased cases (2304, 962, and 820 cases, respectively). However, familial hemophagocytic lymphohistiocytosis exhibited the highest mortality rate (49.0 %). The most common causes of death were infections, transplant-related mortality and non-infectious pulmonary complications, (3429, 2749, and 1141 cases), respectively. Among infectious causes of death, COVID-19 infection accounted for 10.8 % (370 cases). Conclusion: This study identifies specific types of IEI with the highest mortality rates and numbers, alongside immune component defects most strongly associated with increased mortality. Patients with immune dysregulation, defects in cellular immunity, and phagocyte function were particularly linked to higher mortality rates, underscoring the urgent need for improved management strategies for these IEIs. © 2025 Elsevier B.V., All rights reserved.

Style	Citing Format
MLA	Fekrvand S, et al.. "Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis." Mutation Research - Reviews in Mutation Research, vol. 796, no. , 2025, pp. -.
APA	Fekrvand S, Esfahani ZH, Yarahmadi M, Saeediboroujeni A, Salehi H, Hakimelahi A, Alamsihashiani A, Rahmati M, Afsharqasemloo S, Namehgoshayfard NNG, Monfared FT, Afkham EK, Fathi N, Shad TM, Babaha F, Nazari F, Nirouei M, Farid AS, Sanadgol N, ... Yazdani R (2025). Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis. Mutation Research - Reviews in Mutation Research, 796(), -.
Chicago	Fekrvand S, Esfahani ZH, Yarahmadi M, Saeediboroujeni A, Salehi H, Hakimelahi A, Alamsihashiani A, et al.. "Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis." Mutation Research - Reviews in Mutation Research 796, no. (2025): -.
Harvard	Fekrvand S et al. (2025) 'Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis', Mutation Research - Reviews in Mutation Research, 796(), pp. -.
Vancouver	Fekrvand S, Esfahani ZH, Yarahmadi M, Saeediboroujeni A, Salehi H, Hakimelahi A, et al.. Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis. Mutation Research - Reviews in Mutation Research. 2025;796():-.
BibTex	@article{ author = {Fekrvand S and Esfahani ZH and Yarahmadi M and Saeediboroujeni A and Salehi H and Hakimelahi A and Alamsihashiani A and Rahmati M and Afsharqasemloo S and Namehgoshayfard NNG and Monfared FT and Afkham EK and Fathi N and Shad TM and Babaha F and Nazari F and Nirouei M and Farid AS and Sanadgol N and Rafiemanesh H and Marzbali MY and Hassanpour G and Olbrich P and Condinoneto A and Morio T and Gennery AR and Meyts I and Ochs HD and Abolhassani H and Rezaei N and Yazdani R}, title = {Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis}, journal = {Mutation Research - Reviews in Mutation Research}, volume = {796}, number = {}, pages = {-}, year = {2025} }
RIS	TY - JOUR AU - Fekrvand S AU - Esfahani ZH AU - Yarahmadi M AU - Saeediboroujeni A AU - Salehi H AU - Hakimelahi A AU - Alamsihashiani A AU - Rahmati M AU - Afsharqasemloo S AU - Namehgoshayfard NNG AU - Monfared FT AU - Afkham EK AU - Fathi N AU - Shad TM AU - Babaha F AU - Nazari F AU - Nirouei M AU - Farid AS AU - Sanadgol N AU - Rafiemanesh H AU - Marzbali MY AU - Hassanpour G AU - Olbrich P AU - Condinoneto A AU - Morio T AU - Gennery AR AU - Meyts I AU - Ochs HD AU - Abolhassani H AU - Rezaei N AU - Yazdani R TI - Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis JO - Mutation Research - Reviews in Mutation Research VL - 796 IS - SP - EP - PY - 2025 ER -

Style

Citing Format

MLA

Fekrvand S, et al.. "Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis." Mutation Research - Reviews in Mutation Research, vol. 796, no. , 2025, pp. -.

APA

Fekrvand S, Esfahani ZH, Yarahmadi M, Saeediboroujeni A, Salehi H, Hakimelahi A, Alamsihashiani A, Rahmati M, Afsharqasemloo S, Namehgoshayfard NNG, Monfared FT, Afkham EK, Fathi N, Shad TM, Babaha F, Nazari F, Nirouei M, Farid AS, Sanadgol N, ... Yazdani R (2025). Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis. Mutation Research - Reviews in Mutation Research, 796(), -.

Chicago

Fekrvand S, Esfahani ZH, Yarahmadi M, Saeediboroujeni A, Salehi H, Hakimelahi A, Alamsihashiani A, et al.. "Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis." Mutation Research - Reviews in Mutation Research 796, no. (2025): -.

Harvard

Fekrvand S et al. (2025) 'Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis', Mutation Research - Reviews in Mutation Research, 796(), pp. -.

Vancouver

Fekrvand S, Esfahani ZH, Yarahmadi M, Saeediboroujeni A, Salehi H, Hakimelahi A, et al.. Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis. Mutation Research - Reviews in Mutation Research. 2025;796():-.

BibTex

@article{ author = {Fekrvand S and Esfahani ZH and Yarahmadi M and Saeediboroujeni A and Salehi H and Hakimelahi A and Alamsihashiani A and Rahmati M and Afsharqasemloo S and Namehgoshayfard NNG and Monfared FT and Afkham EK and Fathi N and Shad TM and Babaha F and Nazari F and Nirouei M and Farid AS and Sanadgol N and Rafiemanesh H and Marzbali MY and Hassanpour G and Olbrich P and Condinoneto A and Morio T and Gennery AR and Meyts I and Ochs HD and Abolhassani H and Rezaei N and Yazdani R}, title = {Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis}, journal = {Mutation Research - Reviews in Mutation Research}, volume = {796}, number = {}, pages = {-}, year = {2025} }

RIS

TY - JOUR
AU - Fekrvand S
AU - Esfahani ZH
AU - Yarahmadi M
AU - Saeediboroujeni A
AU - Salehi H
AU - Hakimelahi A
AU - Alamsihashiani A
AU - Rahmati M
AU - Afsharqasemloo S
AU - Namehgoshayfard NNG
AU - Monfared FT
AU - Afkham EK
AU - Fathi N
AU - Shad TM
AU - Babaha F
AU - Nazari F
AU - Nirouei M
AU - Farid AS
AU - Sanadgol N
AU - Rafiemanesh H
AU - Marzbali MY
AU - Hassanpour G
AU - Olbrich P
AU - Condinoneto A
AU - Morio T
AU - Gennery AR
AU - Meyts I
AU - Ochs HD
AU - Abolhassani H
AU - Rezaei N
AU - Yazdani R
TI - Mortality Rate and Causes of Death in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis
JO - Mutation Research - Reviews in Mutation Research
VL - 796
IS -
SP -
EP -
PY - 2025
ER -

Science Communicator Platform

Authors

Abstract