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Open Access 01-12-2023 | Immunodeficiency | Research

Assessment of the first presentations of common variable immunodeficiency in a large cohort of patients

Authors: Hossein Esmaeilzadeh, Armita Jokar-Derisi, Amir Hossein Hassani, Reza Yazdani, Samaneh Delavari, Hassan Abolhassani, Negar Mortazavi, Aida Askarisarvestani

Published in: BMC Immunology | Issue 1/2023

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Abstract

Background

Common Variable Immunodeficiency (CVID) is a primary immunodeficiency syndrome resulting in recurrent infections, autoimmunity, and granulomatous manifestations.

Methods and materials

This retrospective study was conducted on an Iranian national registry of immunodeficient patients from 2010 to 2021. The frequency of first presentations of CVID and its association with sex, age of onset, and family history of CVID was evaluated.

Results

A total of 383 patients entered the study, 164 of whom were female, and the rest were male. The mean age of the patients was 25.3 ± 14.5 years. The most frequent first presentations of CVID were pneumonia (36.8%) and diarrhea (19.1%). Patient sex, age of onset, and family history did not make significant differences in first presentations of this disease.

Conclusion

pneumonia is the most common first presentation of CVID. Family history of CVID, the age of symptom onset, and sex made no differences in the first presentations of CVID.
Literature
1.
go back to reference Szczawinska-Poplonyk A, Schwartzmann E, Bukowska-Olech E, Biernat M, Gattner S, Korobacz T, Nowicki F, Wiczuk-Wiczewska M. The pediatric common variable immunodeficiency - from genetics to therapy: a review. Eur J Pediatr. 2022 Apr;181(4):1371–83. https://doi.org/10.1007/s00431-021-04287-6. Epub 2021 Dec 23. PMID: 34939152; PMCID: PMC8964589. Szczawinska-Poplonyk A, Schwartzmann E, Bukowska-Olech E, Biernat M, Gattner S, Korobacz T, Nowicki F, Wiczuk-Wiczewska M. The pediatric common variable immunodeficiency - from genetics to therapy: a review. Eur J Pediatr. 2022 Apr;181(4):1371–83. https://​doi.​org/​10.​1007/​s00431-021-04287-6. Epub 2021 Dec 23. PMID: 34939152; PMCID: PMC8964589.
2.
go back to reference Bagheri Y, Vosughi A, Azizi G, Yazdani R, Kiaee F, Hafezi N, Alimorad S, Khoshmirsafa M, Seif F, Hassanpour G, Abolhassani H, Aghamohammadi A. Comparison of clinical and immunological features and mortality in common variable immunodeficiency and agammaglobulinemia patients. Immunol Lett. 2019 Jun;210:55–62. doi: 10.1016/j.imlet.2019.05.001. Epub 2019 May 3. PMID: 31059734. Bagheri Y, Vosughi A, Azizi G, Yazdani R, Kiaee F, Hafezi N, Alimorad S, Khoshmirsafa M, Seif F, Hassanpour G, Abolhassani H, Aghamohammadi A. Comparison of clinical and immunological features and mortality in common variable immunodeficiency and agammaglobulinemia patients. Immunol Lett. 2019 Jun;210:55–62. doi: 10.1016/j.imlet.2019.05.001. Epub 2019 May 3. PMID: 31059734.
3.
go back to reference Modell V, Orange JS, Quinn J, Modell F. Global report on primary immunodeficiencies: 2018 update from the Jeffrey Modell Centers Network on disease classification, regional trends, treatment modalities, and physician reported outcomes. Immunol Res. 2018 Jun;66(3):367–380. doi: https://doi.org/10.1007/s12026-018-8996-5. PMID: 29744770. Modell V, Orange JS, Quinn J, Modell F. Global report on primary immunodeficiencies: 2018 update from the Jeffrey Modell Centers Network on disease classification, regional trends, treatment modalities, and physician reported outcomes. Immunol Res. 2018 Jun;66(3):367–380. doi: https://​doi.​org/​10.​1007/​s12026-018-8996-5. PMID: 29744770.
4.
go back to reference Odnoletkova I, Kindle G, Quinti I, Grimbacher B, Knerr V, Gathmann B, Ehl S, Mahlaoui N, Van Wilder P, Bogaerts K, de Vries E. ; Plasma Protein Therapeutics Association (PPTA) Taskforce. The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data. Orphanet J Rare Dis. 2018 Nov 12;13(1):201. doi: https://doi.org/10.1186/s13023-018-0941-0. PMID: 30419968; PMCID: PMC6233554. Odnoletkova I, Kindle G, Quinti I, Grimbacher B, Knerr V, Gathmann B, Ehl S, Mahlaoui N, Van Wilder P, Bogaerts K, de Vries E. ; Plasma Protein Therapeutics Association (PPTA) Taskforce. The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data. Orphanet J Rare Dis. 2018 Nov 12;13(1):201. doi: https://​doi.​org/​10.​1186/​s13023-018-0941-0. PMID: 30419968; PMCID: PMC6233554.
5.
go back to reference Zainaldain H, Rizvi FS, Rafiemanesh H, Alizadeh M, Jamee M, Mohammadi S, Kiaee F, Mohammadi H, Babaie F, Yazdani R, Abolhassani H, Aghamohammadi A, Azizi G. Infectious complications reporting in common variable immunodeficiency: a systematic review and Meta-analysis. Oman Med J 2020 Jul 30;35(4):e157. doi: https://doi.org/10.5001/omj.2020.64. PMID: 32802416; PMCID: PMC7417520. Zainaldain H, Rizvi FS, Rafiemanesh H, Alizadeh M, Jamee M, Mohammadi S, Kiaee F, Mohammadi H, Babaie F, Yazdani R, Abolhassani H, Aghamohammadi A, Azizi G. Infectious complications reporting in common variable immunodeficiency: a systematic review and Meta-analysis. Oman Med J 2020 Jul 30;35(4):e157. doi: https://​doi.​org/​10.​5001/​omj.​2020.​64. PMID: 32802416; PMCID: PMC7417520.
6.
go back to reference Janssen LMA, van der Flier M, de Vries E. Lessons Learned From the Clinical Presentation of Common Variable Immunodeficiency Disorders: A Systematic Review and Meta-Analysis. Front Immunol. 2021 Mar 23;12:620709. doi: https://doi.org/10.3389/fimmu.2021.620709. PMID: 33833753; PMCID: PMC8021796. Janssen LMA, van der Flier M, de Vries E. Lessons Learned From the Clinical Presentation of Common Variable Immunodeficiency Disorders: A Systematic Review and Meta-Analysis. Front Immunol. 2021 Mar 23;12:620709. doi: https://​doi.​org/​10.​3389/​fimmu.​2021.​620709. PMID: 33833753; PMCID: PMC8021796.
7.
go back to reference Feuille EJ, Anooshiravani N, Sullivan KE, Fuleihan RL, Cunningham-Rundles C. Autoimmune Cytopenias and Associated Conditions in CVID: a Report from the USIDNET Registry. J Clin Immunol. 2018 Jan;38(1):28–34. https://doi.org/10.1007/s10875-017-0456-9. Epub 2017 Oct 28. PMID: 29080979; PMCID: PMC5743637. Feuille EJ, Anooshiravani N, Sullivan KE, Fuleihan RL, Cunningham-Rundles C. Autoimmune Cytopenias and Associated Conditions in CVID: a Report from the USIDNET Registry. J Clin Immunol. 2018 Jan;38(1):28–34. https://​doi.​org/​10.​1007/​s10875-017-0456-9. Epub 2017 Oct 28. PMID: 29080979; PMCID: PMC5743637.
8.
go back to reference Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, Scheible R, Rusch S, Gasteiger LM, Grimbacher B, Mahlaoui N, Ehl S, ESID Registry Working Party and collaborators. The european Society for Immunodeficiencies (ESID) Registry Working Definitions for the clinical diagnosis of inborn errors of immunity. J Allergy Clin Immunol Pract. 2019 Jul-Aug;7(6):1763–70. Epub 2019 Feb 15. PMID: 30776527. Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, Scheible R, Rusch S, Gasteiger LM, Grimbacher B, Mahlaoui N, Ehl S, ESID Registry Working Party and collaborators. The european Society for Immunodeficiencies (ESID) Registry Working Definitions for the clinical diagnosis of inborn errors of immunity. J Allergy Clin Immunol Pract. 2019 Jul-Aug;7(6):1763–70. Epub 2019 Feb 15. PMID: 30776527.
9.
go back to reference Abolhassani H, Kiaee F, Tavakol M, Chavoshzadeh Z, Mahdaviani SA, Momen T, Yazdani R, Azizi G, Habibi S, Gharagozlou M, Movahedi M, Hamidieh AA, Behniafard N, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Shirkani A, Amin R, Aleyasin S, Faridhosseini R, Jabbari-Azad F, Mohammadzadeh I, Ghaffari J, Shafiei A, Kalantari A, Mansouri M, Mesdaghi M, Babaie D, Ahanchian H, Khoshkhui M, Soheili H, Eslamian MH, Cheraghi T, Dabbaghzadeh A, Tavassoli M, Kalmarzi RN, Mortazavi SH, Kashef S, Esmaeilzadeh H, Tafaroji J, Khalili A, Zandieh F, Sadeghi-Shabestari M, Darougar S, Behmanesh F, Akbari H, Zandkarimi M, Abolnezhadian F, Fayezi A, Moghtaderi M, Ahmadiafshar A, Shakerian B, Sajedi V, Taghvaei B, Safari M, Heidarzadeh M, Ghalebaghi B, Fathi SM, Darabi B, Bazregari S, Bazargan N, Fallahpour M, Khayatzadeh A, Javahertrash N, Bashardoust B, Zamani M, Mohsenzadeh A, Ebrahimi S, Sharafian S, Vosughimotlagh A, Tafakoridelbari M, Rahimi M, Ashournia P, Razaghian A, Rezaei A, Mamishi S, Parvaneh N, Rezaei N, Hammarström L, Aghamohammadi A. Fourth update on the Iranian National Registry of primary immunodeficiencies: integration of molecular diagnosis. J Clin Immunol. 2018 Oct;38(7):816–32. https://doi.org/10.1007/s10875-018-0556-1. Epub 2018 Oct 9. PMID: 30302726. Abolhassani H, Kiaee F, Tavakol M, Chavoshzadeh Z, Mahdaviani SA, Momen T, Yazdani R, Azizi G, Habibi S, Gharagozlou M, Movahedi M, Hamidieh AA, Behniafard N, Nabavi M, Bemanian MH, Arshi S, Molatefi R, Sherkat R, Shirkani A, Amin R, Aleyasin S, Faridhosseini R, Jabbari-Azad F, Mohammadzadeh I, Ghaffari J, Shafiei A, Kalantari A, Mansouri M, Mesdaghi M, Babaie D, Ahanchian H, Khoshkhui M, Soheili H, Eslamian MH, Cheraghi T, Dabbaghzadeh A, Tavassoli M, Kalmarzi RN, Mortazavi SH, Kashef S, Esmaeilzadeh H, Tafaroji J, Khalili A, Zandieh F, Sadeghi-Shabestari M, Darougar S, Behmanesh F, Akbari H, Zandkarimi M, Abolnezhadian F, Fayezi A, Moghtaderi M, Ahmadiafshar A, Shakerian B, Sajedi V, Taghvaei B, Safari M, Heidarzadeh M, Ghalebaghi B, Fathi SM, Darabi B, Bazregari S, Bazargan N, Fallahpour M, Khayatzadeh A, Javahertrash N, Bashardoust B, Zamani M, Mohsenzadeh A, Ebrahimi S, Sharafian S, Vosughimotlagh A, Tafakoridelbari M, Rahimi M, Ashournia P, Razaghian A, Rezaei A, Mamishi S, Parvaneh N, Rezaei N, Hammarström L, Aghamohammadi A. Fourth update on the Iranian National Registry of primary immunodeficiencies: integration of molecular diagnosis. J Clin Immunol. 2018 Oct;38(7):816–32. https://​doi.​org/​10.​1007/​s10875-018-0556-1. Epub 2018 Oct 9. PMID: 30302726.
10.
go back to reference Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999 Dec;93(3):190-7. doi: https://doi.org/10.1006/clim.1999.4799. PMID: 10600329. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999 Dec;93(3):190-7. doi: https://​doi.​org/​10.​1006/​clim.​1999.​4799. PMID: 10600329.
13.
go back to reference Baloh C, Reddy A, Henson M, Prince K, Buckley R, Lugar P. 30-Year Review of Pediatric- and adult-onset CVID: clinical Correlates and Prognostic indicators. J Clin Immunol. 2019 Oct;39(7):678–87. https://doi.org/10.1007/s10875-019-00674-9. Epub 2019 Aug 3. PMID: 31377970; PMCID: PMC6754754. Baloh C, Reddy A, Henson M, Prince K, Buckley R, Lugar P. 30-Year Review of Pediatric- and adult-onset CVID: clinical Correlates and Prognostic indicators. J Clin Immunol. 2019 Oct;39(7):678–87. https://​doi.​org/​10.​1007/​s10875-019-00674-9. Epub 2019 Aug 3. PMID: 31377970; PMCID: PMC6754754.
14.
go back to reference Westh L, Mogensen TH, Dalgaard LS, Bernth Jensen JM, Katzenstein T, Hansen AE, Larsen OD, Terpling S, Nielsen TL, Larsen CS. Identification and Characterization of a Nationwide Danish Adult Common Variable Immunodeficiency Cohort. Scand J Immunol. 2017 Jun;85(6):450–461. doi: https://doi.org/10.1111/sji.12551. PMID: 28370285. Westh L, Mogensen TH, Dalgaard LS, Bernth Jensen JM, Katzenstein T, Hansen AE, Larsen OD, Terpling S, Nielsen TL, Larsen CS. Identification and Characterization of a Nationwide Danish Adult Common Variable Immunodeficiency Cohort. Scand J Immunol. 2017 Jun;85(6):450–461. doi: https://​doi.​org/​10.​1111/​sji.​12551. PMID: 28370285.
15.
go back to reference Gathmann B, Mahlaoui N, CEREDIH, Gérard L, Oksenhendler E, Warnatz K, Schulze I, Kindle G, Kuijpers TW, Dutch WID, van Beem RT, Guzman D, Workman S, Soler-Palacín P, De Gracia J, Witte T, Schmidt RE, Litzman J, Hlavackova E, Thon V, Borte M, Borte S, Kumararatne D, Feighery C, Longhurst H, Helbert M, Szaflarska A, Sediva A, Belohradsky BH, Jones A, Baumann U, Meyts I, Kutukculer N, Wågström P, Galal NM, Roesler J, Farmaki E, Zinovieva N, Ciznar P, Papadopoulou-Alataki E, Bienemann K, Velbri S, Panahloo Z, Grimbacher B, European Society for Immunodeficiencies Registry Working Party. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014 Jul;134(1):116–26. https://doi.org/10.1016/j.jaci.2013.12.1077. Epub 2014 Feb 28. PMID: 24582312. Gathmann B, Mahlaoui N, CEREDIH, Gérard L, Oksenhendler E, Warnatz K, Schulze I, Kindle G, Kuijpers TW, Dutch WID, van Beem RT, Guzman D, Workman S, Soler-Palacín P, De Gracia J, Witte T, Schmidt RE, Litzman J, Hlavackova E, Thon V, Borte M, Borte S, Kumararatne D, Feighery C, Longhurst H, Helbert M, Szaflarska A, Sediva A, Belohradsky BH, Jones A, Baumann U, Meyts I, Kutukculer N, Wågström P, Galal NM, Roesler J, Farmaki E, Zinovieva N, Ciznar P, Papadopoulou-Alataki E, Bienemann K, Velbri S, Panahloo Z, Grimbacher B, European Society for Immunodeficiencies Registry Working Party. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014 Jul;134(1):116–26. https://​doi.​org/​10.​1016/​j.​jaci.​2013.​12.​1077. Epub 2014 Feb 28. PMID: 24582312.
Metadata
Title
Assessment of the first presentations of common variable immunodeficiency in a large cohort of patients
Authors
Hossein Esmaeilzadeh
Armita Jokar-Derisi
Amir Hossein Hassani
Reza Yazdani
Samaneh Delavari
Hassan Abolhassani
Negar Mortazavi
Aida Askarisarvestani
Publication date
01-12-2023
Publisher
BioMed Central
Published in
BMC Immunology / Issue 1/2023
Electronic ISSN: 1471-2172
DOI
https://doi.org/10.1186/s12865-023-00545-4

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