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Common Variable Immunodeficiency

MedGen UID:
423584
•Concept ID:
C2936664
•
Disease or Syndrome
Synonyms: Acquired Hypogammaglobulinemia; Acquired Hypogammaglobulinemias; Hypogammaglobulinemia, Acquired; Hypogammaglobulinemias, Acquired

Definition

A primary immunodeficiency characterized by low levels or absence of all the immunoglobulin classes and lack of B-lymphocytes or plasma cells. It results in recurrent bacterial infections. Complications include autoimmune phenomena and cancer development. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCommon Variable Immunodeficiency

Professional guidelines

PubMed

Treatment of inflammatory complications in common variable immunodeficiency (CVID): current concepts and future perspectives.
Fevang B
Expert Rev Clin Immunol 2023 Jun;19(6):627-638. Epub 2023 Apr 6 doi: 10.1080/1744666X.2023.2198208. PMID: 36996348
AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review.
Green PHR, Paski S, Ko CW, Rubio-Tapia A
Gastroenterology 2022 Nov;163(5):1461-1469. Epub 2022 Sep 19 doi: 10.1053/j.gastro.2022.07.086. PMID: 36137844
Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.
Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A
J Investig Allergol Clin Immunol 2020;30(1):14-34. Epub 2019 Feb 11 doi: 10.18176/jiaci.0388. PMID: 30741636

Recent clinical studies

Etiology

Common Variable Immunodeficiency.
Remiker A, Bolling K, Verbsky J
Med Clin North Am 2024 Jan;108(1):107-121. Epub 2023 Jul 21 doi: 10.1016/j.mcna.2023.06.012. PMID: 37951645
Autoimmune Cytopenias in Common Variable Immunodeficiency Are a Diagnostic and Therapeutic Conundrum: An Update.
Chawla S, Barman P, Tyagi R, Jindal AK, Sharma S, Rawat A, Singh S
Front Immunol 2022;13:869466. Epub 2022 Jun 20 doi: 10.3389/fimmu.2022.869466. PMID: 35795667Free PMC Article
Common Variable Immunodeficiency and Gastric Malignancies.
Leone P, Vacca A, Dammacco F, Racanelli V
Int J Mol Sci 2018 Feb 2;19(2) doi: 10.3390/ijms19020451. PMID: 29393912Free PMC Article
Common Variable Immunodeficiency and Liver Involvement.
Song J, Lleo A, Yang GX, Zhang W, Bowlus CL, Gershwin ME, Leung PSC
Clin Rev Allergy Immunol 2018 Dec;55(3):340-351. doi: 10.1007/s12016-017-8638-z. PMID: 28785926Free PMC Article
International Consensus Document (ICON): Common Variable Immunodeficiency Disorders.
Bonilla FA, Barlan I, Chapel H, Costa-Carvalho BT, Cunningham-Rundles C, de la Morena MT, Espinosa-Rosales FJ, Hammarström L, Nonoyama S, Quinti I, Routes JM, Tang ML, Warnatz K
J Allergy Clin Immunol Pract 2016 Jan-Feb;4(1):38-59. Epub 2015 Nov 7 doi: 10.1016/j.jaip.2015.07.025. PMID: 26563668Free PMC Article

Diagnosis

Common Variable Immunodeficiency.
Remiker A, Bolling K, Verbsky J
Med Clin North Am 2024 Jan;108(1):107-121. Epub 2023 Jul 21 doi: 10.1016/j.mcna.2023.06.012. PMID: 37951645
Common Variable Immunodeficiency: More Pathways than Roads to Rome.
Peng XP, Caballero-Oteyza A, Grimbacher B
Annu Rev Pathol 2023 Jan 24;18:283-310. Epub 2022 Oct 20 doi: 10.1146/annurev-pathmechdis-031521-024229. PMID: 36266261
Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.
Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A
J Investig Allergol Clin Immunol 2020;30(1):14-34. Epub 2019 Feb 11 doi: 10.18176/jiaci.0388. PMID: 30741636
British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders.
Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, Longhurst HJ, Patel SY, Renzoni EA, Sander CR, Avery GR, Babar JL, Buckland MS, Burns S, Egner W, Gompels MM, Gordins P, Haddock JA, Hart SP, Hayman GR, Herriot R, Hoyles RK, Huissoon AP, Jacob J, Nicholson AG, Rassl DM, Sargur RB, Savic S, Seneviratne SL, Sheaff M, Vaitla PM, Walters GI, Whitehouse JL, Wright PA, Condliffe AM
J Allergy Clin Immunol Pract 2017 Jul-Aug;5(4):938-945. Epub 2017 Mar 25 doi: 10.1016/j.jaip.2017.01.021. PMID: 28351785
International Consensus Document (ICON): Common Variable Immunodeficiency Disorders.
Bonilla FA, Barlan I, Chapel H, Costa-Carvalho BT, Cunningham-Rundles C, de la Morena MT, Espinosa-Rosales FJ, Hammarström L, Nonoyama S, Quinti I, Routes JM, Tang ML, Warnatz K
J Allergy Clin Immunol Pract 2016 Jan-Feb;4(1):38-59. Epub 2015 Nov 7 doi: 10.1016/j.jaip.2015.07.025. PMID: 26563668Free PMC Article

Therapy

Treatment of inflammatory complications in common variable immunodeficiency (CVID): current concepts and future perspectives.
Fevang B
Expert Rev Clin Immunol 2023 Jun;19(6):627-638. Epub 2023 Apr 6 doi: 10.1080/1744666X.2023.2198208. PMID: 36996348
Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review.
Lamers OAC, Smits BM, Leavis HL, de Bree GJ, Cunningham-Rundles C, Dalm VASH, Ho HE, Hurst JR, IJspeert H, Prevaes SMPJ, Robinson A, van Stigt AC, Terheggen-Lagro S, van de Ven AAJM, Warnatz K, van de Wijgert JHHM, van Montfrans J
Front Immunol 2021;12:606099. Epub 2021 Apr 15 doi: 10.3389/fimmu.2021.606099. PMID: 33936030Free PMC Article
Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.
Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A
J Investig Allergol Clin Immunol 2020;30(1):14-34. Epub 2019 Feb 11 doi: 10.18176/jiaci.0388. PMID: 30741636
Common Variable Immunodeficiency and Gastric Malignancies.
Leone P, Vacca A, Dammacco F, Racanelli V
Int J Mol Sci 2018 Feb 2;19(2) doi: 10.3390/ijms19020451. PMID: 29393912Free PMC Article
The BAFF/APRIL system: emerging functions beyond B cell biology and autoimmunity.
Vincent FB, Saulep-Easton D, Figgett WA, Fairfax KA, Mackay F
Cytokine Growth Factor Rev 2013 Jun;24(3):203-15. Epub 2013 May 15 doi: 10.1016/j.cytogfr.2013.04.003. PMID: 23684423Free PMC Article

Prognosis

Inborn Errors of Immunity and Autoimmune Disease.
Gray PE, David C
J Allergy Clin Immunol Pract 2023 Jun;11(6):1602-1622. Epub 2023 Apr 28 doi: 10.1016/j.jaip.2023.04.018. PMID: 37119983
Treatment of inflammatory complications in common variable immunodeficiency (CVID): current concepts and future perspectives.
Fevang B
Expert Rev Clin Immunol 2023 Jun;19(6):627-638. Epub 2023 Apr 6 doi: 10.1080/1744666X.2023.2198208. PMID: 36996348
Agammaglobulinemia: from X-linked to Autosomal Forms of Disease.
Cardenas-Morales M, Hernandez-Trujillo VP
Clin Rev Allergy Immunol 2022 Aug;63(1):22-35. Epub 2021 Jul 9 doi: 10.1007/s12016-021-08870-5. PMID: 34241796Free PMC Article
Lymphocytic Esophagitis: Current Understanding and Controversy.
Pittman ME
Am J Surg Pathol 2022 Jan 1;46(1):e55-e63. doi: 10.1097/PAS.0000000000001667. PMID: 33481383
Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review.
Lamers OAC, Smits BM, Leavis HL, de Bree GJ, Cunningham-Rundles C, Dalm VASH, Ho HE, Hurst JR, IJspeert H, Prevaes SMPJ, Robinson A, van Stigt AC, Terheggen-Lagro S, van de Ven AAJM, Warnatz K, van de Wijgert JHHM, van Montfrans J
Front Immunol 2021;12:606099. Epub 2021 Apr 15 doi: 10.3389/fimmu.2021.606099. PMID: 33936030Free PMC Article

Clinical prediction guides

Treatment of inflammatory complications in common variable immunodeficiency (CVID): current concepts and future perspectives.
Fevang B
Expert Rev Clin Immunol 2023 Jun;19(6):627-638. Epub 2023 Apr 6 doi: 10.1080/1744666X.2023.2198208. PMID: 36996348
Autoimmune Cytopenias in Common Variable Immunodeficiency Are a Diagnostic and Therapeutic Conundrum: An Update.
Chawla S, Barman P, Tyagi R, Jindal AK, Sharma S, Rawat A, Singh S
Front Immunol 2022;13:869466. Epub 2022 Jun 20 doi: 10.3389/fimmu.2022.869466. PMID: 35795667Free PMC Article
Agammaglobulinemia: from X-linked to Autosomal Forms of Disease.
Cardenas-Morales M, Hernandez-Trujillo VP
Clin Rev Allergy Immunol 2022 Aug;63(1):22-35. Epub 2021 Jul 9 doi: 10.1007/s12016-021-08870-5. PMID: 34241796Free PMC Article
Lymphocytic Esophagitis: Current Understanding and Controversy.
Pittman ME
Am J Surg Pathol 2022 Jan 1;46(1):e55-e63. doi: 10.1097/PAS.0000000000001667. PMID: 33481383
British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders.
Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, Longhurst HJ, Patel SY, Renzoni EA, Sander CR, Avery GR, Babar JL, Buckland MS, Burns S, Egner W, Gompels MM, Gordins P, Haddock JA, Hart SP, Hayman GR, Herriot R, Hoyles RK, Huissoon AP, Jacob J, Nicholson AG, Rassl DM, Sargur RB, Savic S, Seneviratne SL, Sheaff M, Vaitla PM, Walters GI, Whitehouse JL, Wright PA, Condliffe AM
J Allergy Clin Immunol Pract 2017 Jul-Aug;5(4):938-945. Epub 2017 Mar 25 doi: 10.1016/j.jaip.2017.01.021. PMID: 28351785

Recent systematic reviews

Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review.
Bintalib HM, van de Ven A, Jacob J, Davidsen JR, Fevang B, Hanitsch LG, Malphettes M, van Montfrans J, Maglione PJ, Milito C, Routes J, Warnatz K, Hurst JR
Front Immunol 2023;14:1190235. Epub 2023 May 8 doi: 10.3389/fimmu.2023.1190235. PMID: 37223103Free PMC Article
Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review.
Lamers OAC, Smits BM, Leavis HL, de Bree GJ, Cunningham-Rundles C, Dalm VASH, Ho HE, Hurst JR, IJspeert H, Prevaes SMPJ, Robinson A, van Stigt AC, Terheggen-Lagro S, van de Ven AAJM, Warnatz K, van de Wijgert JHHM, van Montfrans J
Front Immunol 2021;12:606099. Epub 2021 Apr 15 doi: 10.3389/fimmu.2021.606099. PMID: 33936030Free PMC Article
Autoimmunity in common variable immunodeficiency: a systematic review and meta-analysis.
Rizvi FS, Zainaldain H, Rafiemanesh H, Jamee M, Hossein-Khannazer N, Hamedifar H, Sabzevari A, Yazdani R, Abolhassani H, Aghamohammadi A, Azizi G
Expert Rev Clin Immunol 2020 Dec;16(12):1227-1235. Epub 2020 Dec 7 doi: 10.1080/1744666X.2021.1850272. PMID: 33203275
Bronchiectasis in common variable immunodeficiency: A systematic review and meta-analysis.
Ramzi N, Jamee M, Bakhtiyari M, Rafiemanesh H, Zainaldain H, Tavakol M, Rezaei A, Kalvandi M, Zian Z, Mohammadi H, Jadidi-Niaragh F, Yazdani R, Abolhassani H, Aghamohammadi A, Azizi G
Pediatr Pulmonol 2020 Feb;55(2):292-299. Epub 2019 Dec 13 doi: 10.1002/ppul.24599. PMID: 31833673
Primary immunodeficiency and recalcitrant chronic sinusitis: a systematic review.
Mazza JM, Lin SY
Int Forum Allergy Rhinol 2016 Oct;6(10):1029-1033. Epub 2016 May 17 doi: 10.1002/alr.21789. PMID: 27187624

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