Common Variable Immunodeficiency

Heterogeneous group of immunodeficiency syndromes characterized by hypogammaglobulinemia of most isotypes, variable B-cell defects, and the presence of recurrent bacterial infections.

Also Known As:

Common Variable Hypogammaglobulinemia; Common Variable Immune Deficiency; Hypogammaglobulinemia, Acquired; Immunoglobulin Deficiency, Late-Onset; Acquired Hypogammaglobulinemia; Acquired Hypogammaglobulinemias; Common Variable Hypogammaglobulinemias; Common Variable Immunodeficiencies; Deficiencies, Late-Onset Immunoglobulin; Deficiency, Late-Onset Immunoglobulin; Hypogammaglobulinemia, Common Variable; Hypogammaglobulinemias, Acquired; Hypogammaglobulinemias, Common Variable; Immunodeficiencies, Common Variable; Immunoglobulin Deficiencies, Late-Onset; Immunoglobulin Deficiency, Late Onset; Late-Onset Immunoglobulin Deficiencies; Late-Onset Immunoglobulin Deficiency; Variable Hypogammaglobulinemia, Common; Variable Hypogammaglobulinemias, Common; Immunodeficiency, Common Variable

Networked: 1351 relevant articles (26 outcomes, 70 trials/studies)

Relationship Network

Disease Context: Research Results

Immune System Diseases: 4430
- Immunologic Deficiency Syndromes: 91
  - Common Variable Immunodeficiency: 1351

Related Diseases

1.	Infections
2.	Bruton type agammaglobulinemia
3.	COVID-19
4.	Severe Combined Immunodeficiency (Bare Lymphocyte Syndrome)
5.	Hyperplasia

Experts

1.	Cunningham-Rundles, Charlotte: 35 articles (01/2022 - 06/2002)
2.	Aghamohammadi, Asghar: 31 articles (02/2022 - 09/2003)
3.	Rezaei, Nima: 26 articles (01/2020 - 01/2004)
4.	Grimbacher, Bodo: 20 articles (01/2023 - 12/2005)
5.	Spadaro, Giuseppe: 18 articles (10/2023 - 05/2007)
6.	Aukrust, Pål: 18 articles (01/2023 - 06/2003)
7.	Quinti, Isabella: 17 articles (01/2023 - 04/2003)
8.	Fevang, Børre: 15 articles (01/2023 - 02/2009)
9.	Plebani, Alessandro: 15 articles (01/2023 - 03/2003)
10.	Warnatz, Klaus: 15 articles (01/2023 - 03/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Common Variable Immunodeficiency:

1.	Immunoglobulins (Immunoglobulin)IBA 12/15/2007 - "The present study compared two different systems of classification of patients with common variable immunodeficiency (CVID); one based on in vitro immunoglobulin biosynthesis; and another on CD4-naïve cell counts. " 01/01/2023 - "Fecal Immunoglobulin Levels as a Modifier of the Gut Microbiome in Patients with Common Variable Immunodeficiency." 11/01/2022 - "Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies characterized by impaired immunoglobulin production and dysregulated immune response. " 03/01/2022 - "Common variable immune deficiency (CVID) encompasses a variety of diseases characterized by disturbed immunoglobulin (Ig) production and various immune dysregulations. " 01/01/2022 - "On further investigation, he was found to have deficiencies in immunoglobulins consistent with common variable immunodeficiency (CVID). "
2.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2011 - "Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency." 06/15/2018 - "This prospective, open-label, non-randomised, multicentre, phase III trial investigated the pharmacokinetics of a new 10% liquid IVIG product (panzyga®; Octapharma) in 51 patients aged 2-75 years with common variable immunodeficiency (n = 43) or X-linked agammaglobulinaemia (n = 8). " 12/01/2014 - "Recent studies suggest that in vitro exposure of human B cells to IVIG determines functional changes reminiscent of anergy and that IVIG treatment of patients with common variable immunodeficiency (CVID) induces in B cells ERK activation, a feature of anergy. " 06/11/2022 - "He was treated regularly with intravenous immunoglobulin from the age of 10 years with a diagnosis of common variable immune deficiency. " 01/01/2020 - "We report the case of a 9-yr-old boy diagnosed with common variable immunodeficiency at the age of 3, who is under monthly intravenous immunoglobulin. "
3.	VaccinesIBA 01/01/2023 - "Our aim is to test humoral and cell-mediated response after three doses of BNT162b vaccine in two cohorts of fragile patients: Common Variable Immunodeficiency (CVID) patients and Kidney Transplant Recipients (KTR) patients compared to healthy donors. " 01/01/2023 - " we describe a case of a 50-year-old man with common variable immune deficiency, persistently infected with a neurovirulent vaccine-derived type 2 poliovirus following vaccination in childhood. " 01/01/2022 - "Easy approach to detect cell immunity to COVID vaccines in common variable immunodeficiency patients." 01/01/2022 - "Immunogenicity of Anti-SARS-CoV-2 Vaccines in Common Variable Immunodeficiency." 01/01/2022 - "Testing for common variable immunodeficiency (CVID) requires evaluation of specific antibody responses to vaccines. "
4.	Rituximab (Mabthera)FDA Link 11/01/2022 - "Risk factors for poor antibody responses included diagnosis of common variable immunodeficiency, presence of autoimmune comorbidities, and use of rituximab. " 01/01/2019 - "Immunosuppressive therapy with rituximab in common variable immunodeficiency." 12/01/2018 - "The case report presented suggests possible efficacy and safety of rituximab monotherapy in patients with a complicated form of common variable immunodeficiency. " 09/01/2014 - "Two of these patients were diagnosed with common variable immunodeficiency before rituximab treatment. " 09/01/2006 - "Abnormal repertoires and impaired isotype expression are seen in patients with common variable immunodeficiency, these data suggested that rituximab after APBSCT can affect not only the B-cell quantities, but also the recovery of the B-cell repertoires."
5.	Immunoglobulin G (IgG)IBA 03/01/2002 - "Seventeen patients with antibody immunodeficiency (9 subclass IgG immunodeficiencies, 8 common variable immunodeficiencies) and clinically unambiguous immunodeficiency symptomatology participated in the study with 14 healthy donors. " 05/01/2024 - "Background: The main treatment of common variable immunodeficiency (CVID) is to maintain immunoglobulin G (IgG) levels within the target range. " 01/01/2022 - "Serum IgG1 levels were low and findings in one case were consistent with common variable immunodeficiency (CVID). " 01/01/2021 - "Twenty-nine patients with common variable immunodeficiency or IgG subclass deficiency were vaccinated. " 09/01/2020 - "Common variable immunodeficiency (CVID) is a kind of hypoimmunoglobulinemia of different spectrum with a dominant decrease of IgG with heterogeneity of clinical manifestations. "
6.	AntibodiesIBA 12/01/2023 - "Common variable immunodeficiency (CVID) is an inborn error of immunity characterized by low levels of antibodies. " 05/01/2013 - "Common variable immune deficiency (CVID) is a heterogeneous disease associated with ineffective production of antibodies. " 11/01/2010 - "Common variable immunodeficiency (CVID) is an immunodeficiency characterized by an impaired ability to produce antibodies associated to multiple clinical phenotypes. " 11/01/2006 - "Common variable immunodeficiency (CVID) is a heterogeneous entity characterized by an impaired ability to produce antibodies. " 01/01/2004 - "Common variable immunodeficiency is one of the main antibodies' deficiency syndromes. "
7.	AntigensIBA 04/01/1984 - "This study demonstrates the genetic nature, possibly autosomal dominant inheritance, of common variable immunodeficiency; however the immunodeficiency is not linked to any specific HLA antigen." 05/10/2015 - "Dysfunctional BLK in common variable immunodeficiency perturbs B-cell proliferation and ability to elicit antigen-specific CD4+ T-cell help." 01/01/2011 - "Common variable immunodeficiency (CVID) is a primary immunodeficiency characterised by hypogammaglobulinaemia and antibody deficiency to T dependent and independent antigens. " 01/01/2010 - "Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinaemia and antibody deficiency to both T dependent and independent antigens. " 10/01/2007 - "Our data suggest that TACI dysfunction could contribute to the impaired antibody response to T-dependent antigens in common variable immunodeficiency."
8.	T-Cell Antigen Receptors (T-Cell Receptor)IBA 01/01/1996 - "In this study, we report that differences between T-cell receptor (TCR) V beta gene family usage in CD4+ and CD8+ T cells are significantly greater in a subgroup of patients with common variable immunodeficiency (CVI) and high levels of activated CD8+ T cells (CD8hi CVI) than in controls (P < 0.001). " 01/01/2017 - "We used high throughput sequencing to examine the structure and composition of the T cell receptor β chain in Common Variable Immune Deficiency (CVID). " 01/01/2016 - "We used a triplex real-time polymerase chain reaction (PCR) to classify our common variable immunodeficiency (CVID) patients into distinct groups according to the amount of their T-cell receptor excision circles (TRECs) and κ-deleting recombination excision circles (KRECs). " 01/01/2016 - "Limitation of Simultaneous Analysis of T-Cell Receptor and κ-Deleting Recombination Excision Circles Based on Multiplex Real-Time Polymerase Chain Reaction in Common Variable Immunodeficiency Patients." 11/01/2004 - "Assessing thymopoiesis in patients with common variable immunodeficiency as measured by T-cell receptor excision circles."
9.	Cimetidine (Biomet)FDA LinkGeneric 11/01/1989 - "Common variable immunodeficiency: a family study and therapeutic trial with cimetidine." 07/25/1985 - "Cimetidine and common variable hypogammaglobulinemia." 01/24/1985 - "Modulation of suppressor-cell activity by cimetidine in patients with common variable hypogammaglobulinemia." 06/01/1996 - "Cimetidine, an H2-receptor antagonist, has been used successfully to treat patients with mucocutaneous candidiasis, common variable immunodeficiency, herpes simplex, and herpes zoster because of its immunomodulatory effects. " 03/01/1990 - "Preliminary reports also indicate that cimetidine may offer therapeutic benefits for patients with Varicella zoster and Herpes simplex infections, as well as those suffering from mucocutaneous candidiasis and common variable hypogammaglobulinemia. "
10.	Proteins (Proteins, Gene)FDA Link 01/01/2024 - "Eight of the 11 individuals with severely impaired responses to both PnPS and proteins had common variable immunodeficiency. " 09/01/2022 - "Low levels of the innate immune system proteins MASP-2 and MAp44 in patients with common variable immunodeficiency." 02/01/2022 - "Evaluation of Expression of LRBA and CTLA-4 Proteins in Common Variable Immunodeficiency Patients." 01/01/2021 - "A Pathogenic Missense Variant in NFKB1 Causes Common Variable Immunodeficiency Due to Detrimental Protein Damage." 01/01/2011 - "B-cell defects in common variable immunodeficiency: BCR signaling, protein clustering and hardwired gene mutations."

Therapies and Procedures

1.	Therapeutics 10/29/2023 - "A male patient in his 20s with a medical history of common variable immunodeficiency disorder, non-compliant with therapy, presented to the emergency department with respiratory distress and severe hypoxaemia. " 04/01/2022 - "The pediatric common variable immunodeficiency - from genetics to therapy: a review." 01/01/2022 - "Patients with secondary PAD (eg, B-cell depletion therapy was used) and those with severe primary PAD (eg, common variable immunodeficiency with autoinflammatory complications) had the lowest mean anti-spike antibody levels. " 01/01/2020 - "Personalized therapy for common variable immunodeficiency." 11/01/2019 - "To update the reader on recently proposed common variable immune deficiency (CVID) diagnostic criteria, newly uncovered CVID pathobiology, freshly identified CVID-related genes, and novel CVID therapies. "
2.	Splenectomy 04/01/2013 - "Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients." 01/01/2010 - "[Accessory spleens after splenectomy in a patient with common variable immunodeficiency]." 01/17/2024 - "About 25% of patients with common variable immunodeficiency disease (CVID) have splenomegaly, necessitating sometimes splenectomy whom consequences on the immunological profile of CVID patients have never been studied. " 01/17/2024 - "Influence of Splenomegaly and Splenectomy on the Immune Cell Profile of Patients with Common Variable Immunodeficiency Disease." 10/14/2021 - "Objective: To improve the understanding of splenectomy for treating common variable immunodeficiency complicated with cytopenia. "
3.	Hematopoietic Stem Cell Transplantation 04/01/2015 - "Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency." 01/01/2021 - "Clinical features of a 3-month-old male infant who underwent hematopoietic stem cell transplantation because of an IKZF1 mutation-associated common variable immunodeficiency, AIHA, and pancytopenia are described. " 03/19/2007 - "These new insights will support the understanding of peripheral B cell regeneration after hematopoietic stem cell transplantation or B cell-directed antibody therapy, and the identification of defects in homeostatic or antigen-induced B cell proliferation in patients with common variable immunodeficiency or another antibody deficiency." 10/01/2018 - "These include (1) the role of follicular T cells in the differentiation of B cells and development of optimal antibody responses; (2) impaired nuclear factor κB subunit 1 signaling in the pathogenesis of common variable immunodeficiency, revealing an association between impaired B-cell maturation and development of inflammatory conditions; (3) autoimmune and inflammatory manifestations in patients with PIDDs in T- and B-cell deficiencies, as well as in neutrophil disorders; (4) newly described gene defects causing PIDDs, including exostosin-like 3 (EXTL3), TNF-α-induced protein 3 (TNFAIP3 [A20]), actin-related protein 2/3 complex-subunit 1B (ARPC1B), v-Rel avian reticuloendotheliosis viral oncogene homolog A (RELA), hypoxia upregulated 1 (HYOU1), BTB domain and CNC homolog 2 (BACH2), CD70, and CD55; (5) use of rapamycin and the phosphoinositide 3-kinase inhibitor leniolisib to reduce autoimmunity and regulate B-cell function in the activated phosphoinositide 3-kinase δ syndrome; (6) improved outcomes in hematopoietic stem cell transplantation for severe combined immunodeficiency (SCID) in the last decade, with an overall 2-year survival of 90% in part caused by early diagnosis through implementation of universal newborn screening; (7) demonstration of the efficacy of lentiviral vector-mediated gene therapy for patients with adenosine deaminase-deficient SCID; (8) the promise of gene editing for PIDDs using CRISPR/Cas9 and zinc finger nuclease technology for SCID and chronic granulomatous disease; and (9) the efficacy of thymus transplantation in Europe, although associated with an unexpected high incidence of autoimmunity. "
4.	Transplantation 01/01/2018 - "[Successful cord blood transplantation in a patient with adult-onset common variable immunodeficiency]." 09/01/1998 - "Five patients had acquired immunodeficiency syndrome (AIDS); one had common variable immune deficiency; and 18 were receiving immunosuppressive therapy for heart, lung, or heart-lung (n =15) or bone marrow (n = 2) transplantation and vasculitis (n = 1). " 01/01/2023 - "Recipients transplanted for bronchiectasis in the context of a primary immune deficiency, such as common variable immunodeficiency, are at a high risk of severe infection in post-transplantation leading to poorer long-term outcomes than other transplant indications. " 10/01/2018 - "These include (1) the role of follicular T cells in the differentiation of B cells and development of optimal antibody responses; (2) impaired nuclear factor κB subunit 1 signaling in the pathogenesis of common variable immunodeficiency, revealing an association between impaired B-cell maturation and development of inflammatory conditions; (3) autoimmune and inflammatory manifestations in patients with PIDDs in T- and B-cell deficiencies, as well as in neutrophil disorders; (4) newly described gene defects causing PIDDs, including exostosin-like 3 (EXTL3), TNF-α-induced protein 3 (TNFAIP3 [A20]), actin-related protein 2/3 complex-subunit 1B (ARPC1B), v-Rel avian reticuloendotheliosis viral oncogene homolog A (RELA), hypoxia upregulated 1 (HYOU1), BTB domain and CNC homolog 2 (BACH2), CD70, and CD55; (5) use of rapamycin and the phosphoinositide 3-kinase inhibitor leniolisib to reduce autoimmunity and regulate B-cell function in the activated phosphoinositide 3-kinase δ syndrome; (6) improved outcomes in hematopoietic stem cell transplantation for severe combined immunodeficiency (SCID) in the last decade, with an overall 2-year survival of 90% in part caused by early diagnosis through implementation of universal newborn screening; (7) demonstration of the efficacy of lentiviral vector-mediated gene therapy for patients with adenosine deaminase-deficient SCID; (8) the promise of gene editing for PIDDs using CRISPR/Cas9 and zinc finger nuclease technology for SCID and chronic granulomatous disease; and (9) the efficacy of thymus transplantation in Europe, although associated with an unexpected high incidence of autoimmunity. "
5.	Aftercare (After-Treatment) 01/01/2023 - "After treatment, nsp12-V166A was emerged as a remdesivir resistance and S-L452M in a patient with common variable immunodeficiency. " 09/01/1997 - "We report the case of a patient with common variable immunodeficiency and hepatitis C in whom a sustained biochemical and viral remission was obtained after treatment with interferon."