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Common variable immunodeficiency(CVID)

MedGen UID:
40407
Concept ID:
C0009447
Disease or Syndrome
Synonyms: Common variable agammaglobulinemia; Common variable hypogamma-globulinemia; CVID
SNOMED CT: Common variable agammaglobulinemia (23238000); CVAG (23238000); Common variable immunodeficiency (23238000); CVI - Common variable immunodeficiency (23238000); Late onset immunoglobulin deficiency (23238000); CVID - Common variable immunodeficiency (23238000); Common variable hypogammaglobulinemia (23238000)
 
OMIM®: 240500; 607594
Orphanet: ORPHA1572

Definition

Common variable immune deficiency (CVID) is a disorder that impairs the immune system. People with CVID are highly susceptible to infection from foreign invaders such as bacteria, or more rarely, viruses and often develop recurrent infections, particularly in the lungs, sinuses, and ears. Pneumonia is common in people with CVID. Over time, recurrent infections can lead to chronic lung disease. Affected individuals may also experience infection or inflammation of the gastrointestinal tract, which can cause diarrhea and weight loss. Abnormal accumulation of immune cells causes enlarged lymph nodes (lymphadenopathy) or an enlarged spleen (splenomegaly) in some people with CVID. Immune cells can accumulate in other organs, forming small lumps called granulomas.Approximately 25 percent of people with CVID have an autoimmune disorder, which occurs when the immune system malfunctions and attacks the body's tissues and organs. The blood cells are most frequently affected by autoimmune attacks in CVID; the most commonly occurring autoimmune disorders are immune thrombocytopenia purpura, which is an abnormal bleeding disorder caused by a decrease in platelets, and autoimmune hemolytic anemia, which results in premature destruction of red blood cells. Other autoimmune disorders such as rheumatoid arthritis can occur. Individuals with CVID also have a greater than normal risk of developing certain types of cancer, including a cancer of immune system cells called non-Hodgkin lymphoma and less frequently, stomach (gastric) cancer.People with CVID may start experiencing signs and symptoms of the disorder anytime between childhood and adulthood. The life expectancy of individuals with CVID varies depending on the severity and frequency of illnesses they experience. Most people with CVID live into adulthood.
[from GHR]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCommon variable immunodeficiency
Follow this link to review classifications for Common variable immunodeficiency in Orphanet.

Recent clinical studies

Etiology

Zea-Vera AF, Agudelo-Rojas OL
Colomb Med (Cali) 2015 Jan-Mar;46(1):47-50. Epub 2015 Mar 30 PMID: 26019385Free PMC Article
Compeer EB, Janssen W, van Royen-Kerkhof A, van Gijn M, van Montfrans JM, Boes M
Oncotarget 2015 May 10;6(13):10759-71. doi: 10.18632/oncotarget.3577. PMID: 25926555Free PMC Article
Lougaris V, Ravelli A, Villanacci V, Salemme M, Soresina A, Fuoti M, Lanzarotto F, Lanzini A, Plebani A, Bassotti G
Dig Dis Sci 2015 Aug;60(8):2384-9. Epub 2015 Mar 28 doi: 10.1007/s10620-015-3638-4. [Epub ahead of print] PMID: 25821101
Maggadottir SM, Li J, Glessner JT, Li YR, Wei Z, Chang X, Mentch FD, Thomas KA, Kim CE, Zhao Y, Hou C, Wang F, Jørgensen SF, Perez EE, Sullivan KE, Orange JS, Karlsen TH, Chapel H, Cunningham-Rundles C, Hakonarson H
J Allergy Clin Immunol 2015 Jun;135(6):1569-77. Epub 2015 Feb 10 doi: 10.1016/j.jaci.2014.12.1939. [Epub ahead of print] PMID: 25678086Free PMC Article
Wehr C, Gennery AR, Lindemans C, Schulz A, Hoenig M, Marks R, Recher M, Gruhn B, Holbro A, Heijnen I, Meyer D, Grigoleit G, Einsele H, Baumann U, Witte T, Sykora KW, Goldacker S, Regairaz L, Aksoylar S, Ardeniz Ö, Zecca M, Zdziarski P, Meyts I, Matthes-Martin S, Imai K, Kamae C, Fielding A, Seneviratne S, Mahlaoui N, Slatter MA, Güngör T, Arkwright PD, van Montfrans J, Sullivan KE, Grimbacher B, Cant A, Peter HH, Finke J, Gaspar HB, Warnatz K, Rizzi M; Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiency
J Allergy Clin Immunol 2015 Apr;135(4):988-97.e6. Epub 2015 Jan 14 doi: 10.1016/j.jaci.2014.11.029. [Epub ahead of print] PMID: 25595268

Diagnosis

Rao N, Mackinnon AC, Routes JM
Hum Pathol 2015 Sep;46(9):1306-14. Epub 2015 Jun 1 doi: 10.1016/j.humpath.2015.05.011. [Epub ahead of print] PMID: 26138782Free PMC Article
Compeer EB, Janssen W, van Royen-Kerkhof A, van Gijn M, van Montfrans JM, Boes M
Oncotarget 2015 May 10;6(13):10759-71. doi: 10.18632/oncotarget.3577. PMID: 25926555Free PMC Article
Sadeghi B, Abolhassani H, Naseri A, Rezaei N, Aghamohammadi A
Expert Rev Clin Immunol 2015 May;11(5):681-8. Epub 2015 Mar 25 doi: 10.1586/1744666X.2015.1029457. [Epub ahead of print] PMID: 25804338
Maggadottir SM, Li J, Glessner JT, Li YR, Wei Z, Chang X, Mentch FD, Thomas KA, Kim CE, Zhao Y, Hou C, Wang F, Jørgensen SF, Perez EE, Sullivan KE, Orange JS, Karlsen TH, Chapel H, Cunningham-Rundles C, Hakonarson H
J Allergy Clin Immunol 2015 Jun;135(6):1569-77. Epub 2015 Feb 10 doi: 10.1016/j.jaci.2014.12.1939. [Epub ahead of print] PMID: 25678086Free PMC Article
Wehr C, Gennery AR, Lindemans C, Schulz A, Hoenig M, Marks R, Recher M, Gruhn B, Holbro A, Heijnen I, Meyer D, Grigoleit G, Einsele H, Baumann U, Witte T, Sykora KW, Goldacker S, Regairaz L, Aksoylar S, Ardeniz Ö, Zecca M, Zdziarski P, Meyts I, Matthes-Martin S, Imai K, Kamae C, Fielding A, Seneviratne S, Mahlaoui N, Slatter MA, Güngör T, Arkwright PD, van Montfrans J, Sullivan KE, Grimbacher B, Cant A, Peter HH, Finke J, Gaspar HB, Warnatz K, Rizzi M; Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiency
J Allergy Clin Immunol 2015 Apr;135(4):988-97.e6. Epub 2015 Jan 14 doi: 10.1016/j.jaci.2014.11.029. [Epub ahead of print] PMID: 25595268

Therapy

Zea-Vera AF, Agudelo-Rojas OL
Colomb Med (Cali) 2015 Jan-Mar;46(1):47-50. Epub 2015 Mar 30 PMID: 26019385Free PMC Article
Sadeghi B, Abolhassani H, Naseri A, Rezaei N, Aghamohammadi A
Expert Rev Clin Immunol 2015 May;11(5):681-8. Epub 2015 Mar 25 doi: 10.1586/1744666X.2015.1029457. [Epub ahead of print] PMID: 25804338
Wehr C, Gennery AR, Lindemans C, Schulz A, Hoenig M, Marks R, Recher M, Gruhn B, Holbro A, Heijnen I, Meyer D, Grigoleit G, Einsele H, Baumann U, Witte T, Sykora KW, Goldacker S, Regairaz L, Aksoylar S, Ardeniz Ö, Zecca M, Zdziarski P, Meyts I, Matthes-Martin S, Imai K, Kamae C, Fielding A, Seneviratne S, Mahlaoui N, Slatter MA, Güngör T, Arkwright PD, van Montfrans J, Sullivan KE, Grimbacher B, Cant A, Peter HH, Finke J, Gaspar HB, Warnatz K, Rizzi M; Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiency
J Allergy Clin Immunol 2015 Apr;135(4):988-97.e6. Epub 2015 Jan 14 doi: 10.1016/j.jaci.2014.11.029. [Epub ahead of print] PMID: 25595268
Metzger ML, Michelfelder I, Goldacker S, Melkaoui K, Litzman J, Guzman D, Grimbacher B, Salzer U
Clin Exp Immunol 2015 Feb;179(2):256-64. doi: 10.1111/cei.12459. PMID: 25251245Free PMC Article
Visentini M, Marrapodi R, Conti V, Mitrevski M, Camponeschi A, Lazzeri C, Carbonari M, Catizone A, Quinti I, Fiorilli M
J Allergy Clin Immunol 2014 Aug;134(2):401-10. Epub 2014 May 1 doi: 10.1016/j.jaci.2014.03.017. [Epub ahead of print] PMID: 24792875

Prognosis

Rao N, Mackinnon AC, Routes JM
Hum Pathol 2015 Sep;46(9):1306-14. Epub 2015 Jun 1 doi: 10.1016/j.humpath.2015.05.011. [Epub ahead of print] PMID: 26138782Free PMC Article
Zea-Vera AF, Agudelo-Rojas OL
Colomb Med (Cali) 2015 Jan-Mar;46(1):47-50. Epub 2015 Mar 30 PMID: 26019385Free PMC Article
Arslan S, Ucar R, Yavsan DM, Esen H, Maden E, Reisli I, Calıskaner AZ
J Clin Immunol 2015 May;35(4):344-7. Epub 2015 Mar 14 doi: 10.1007/s10875-015-0151-7. [Epub ahead of print] PMID: 25773572
Wehr C, Gennery AR, Lindemans C, Schulz A, Hoenig M, Marks R, Recher M, Gruhn B, Holbro A, Heijnen I, Meyer D, Grigoleit G, Einsele H, Baumann U, Witte T, Sykora KW, Goldacker S, Regairaz L, Aksoylar S, Ardeniz Ö, Zecca M, Zdziarski P, Meyts I, Matthes-Martin S, Imai K, Kamae C, Fielding A, Seneviratne S, Mahlaoui N, Slatter MA, Güngör T, Arkwright PD, van Montfrans J, Sullivan KE, Grimbacher B, Cant A, Peter HH, Finke J, Gaspar HB, Warnatz K, Rizzi M; Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiency
J Allergy Clin Immunol 2015 Apr;135(4):988-97.e6. Epub 2015 Jan 14 doi: 10.1016/j.jaci.2014.11.029. [Epub ahead of print] PMID: 25595268
Buchbinder D, Baker R, Lee YN, Ravell J, Zhang Y, McElwee J, Nugent D, Coonrod EM, Durtschi JD, Augustine NH, Voelkerding KV, Csomos K, Rosen L, Browne S, Walter JE, Notarangelo LD, Hill HR, Kumánovics A
J Clin Immunol 2015 Feb;35(2):119-24. Epub 2014 Dec 17 doi: 10.1007/s10875-014-0121-5. [Epub ahead of print] PMID: 25516070Free PMC Article

Clinical prediction guides

Indrevær RL, Moskaug JØ, Paur I, Bøhn SK, Jørgensen SF, Blomhoff R, Aukrust P, Fevang B, Blomhoff HK
J Immunol 2015 Sep 15;195(6):2601-11. Epub 2015 Aug 14 doi: 10.4049/jimmunol.1500250. [Epub ahead of print] PMID: 26276871
Abolhassani H, Cheraghi T, Rezaei N, Aghamohammadi A, Hammarström L
J Investig Allergol Clin Immunol 2015;25(3):218-20. PMID: 26182690
Sadeghi B, Abolhassani H, Naseri A, Rezaei N, Aghamohammadi A
Expert Rev Clin Immunol 2015 May;11(5):681-8. Epub 2015 Mar 25 doi: 10.1586/1744666X.2015.1029457. [Epub ahead of print] PMID: 25804338
Metzger ML, Michelfelder I, Goldacker S, Melkaoui K, Litzman J, Guzman D, Grimbacher B, Salzer U
Clin Exp Immunol 2015 Feb;179(2):256-64. doi: 10.1111/cei.12459. PMID: 25251245Free PMC Article
Borte S, Celiksoy MH, Menzel V, Ozkaya O, Ozen FZ, Hammarström L, Yildiran A
Clin Immunol 2014 Oct;154(2):105-11. Epub 2014 Jul 23 doi: 10.1016/j.clim.2014.07.003. [Epub ahead of print] PMID: 25064839

Recent systematic reviews

Borte S, Celiksoy MH, Menzel V, Ozkaya O, Ozen FZ, Hammarström L, Yildiran A
Clin Immunol 2014 Oct;154(2):105-11. Epub 2014 Jul 23 doi: 10.1016/j.clim.2014.07.003. [Epub ahead of print] PMID: 25064839
Hussein K, Rath B, Ludewig B, Kreipe H, Jonigk D
Eur J Cancer 2014 Sep;50(14):2417-24. Epub 2014 Jul 11 doi: 10.1016/j.ejca.2014.06.006. [Epub ahead of print] PMID: 25027306
Salzer U, Warnatz K, Peter HH
Arthritis Res Ther 2012 Sep 24;14(5):223. doi: 10.1186/ar4032. [Epub ahead of print] PMID: 23043756Free PMC Article
Eibel H, Salzer U, Warnatz K
Curr Opin Allergy Clin Immunol 2010 Dec;10(6):526-33. doi: 10.1097/ACI.0b013e32833fea1c. PMID: 20864887
Eisenstein EM, Sneller MC
Ann Allergy 1994 Oct;73(4):285-92; quiz 293-4. PMID: 7943995

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