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Common Variable Immunodeficiency

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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; 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: 832 relevant articles (13 outcomes, 43 trials/studies)

Relationship Network

Disease Context: Research Results

Immune System Diseases: 2064
- Immunologic Deficiency Syndromes: 73
  - Common Variable Immunodeficiency: 832

Related Diseases

1.	Infection
2.	Sinusitis
3.	Inflammation
4.	Pneumonia (Pneumonitis)
5.	Herpes Zoster

Experts

1.	Aghamohammadi, Asghar: 24 articles (07/2014 - 11/2002)
2.	Rezaei, Nima: 19 articles (07/2014 - 11/2002)
3.	Cunningham-Rundles, Charlotte: 17 articles (11/2015 - 06/2002)
4.	Plebani, Alessandro: 13 articles (04/2014 - 03/2003)
5.	Quinti, Isabella: 11 articles (09/2015 - 04/2003)
6.	Grimbacher, Bodo: 11 articles (04/2015 - 12/2005)
7.	Salzer, Ulrich: 9 articles (04/2014 - 12/2005)
8.	Moin, Mostafa: 9 articles (11/2009 - 01/2004)
9.	Pourpak, Zahra: 9 articles (07/2007 - 11/2002)
10.	Aukrust, Pål: 8 articles (09/2015 - 06/2003)

Drugs and Biologics

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

1.	Immunoglobulins (Immunoglobulin)IBA 01/01/2011 - "Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency." 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. " 03/01/2001 - "In this retrospective, nationwide survey data were collected on all patients with common variable immunodeficiency who were receiving immunoglobulin replacement therapy in Finland to study the prediagnostic clinical picture, diagnostic delay, and diagnostic findings. " 10/01/1989 - "Studies in our laboratory demonstrated that the long-term administration of intravenous immune serum globulin in patients with common variable hypogammaglobulinemia produced increased suppressor activity, which resulted in diminished in vitro B cell immunoglobulin synthesis. " 09/01/2015 - "Intravenous immunoglobulin replacement induces an in vivo reduction of inflammatory monocytes and retains the monocyte ability to respond to bacterial stimulation in patients with common variable immunodeficiencies."
2.	Intravenous Immunoglobulins (IVIG)FDA Link 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. " 10/01/1989 - "The present study was undertaken to evaluate the effects of intravenous immune globulin replacement therapy in patients with common variable hypogammaglobulinemia. " 01/01/2015 - "In accord with this, we recently reported that IVIG therapy in patients affected by common variable immunodeficiency (CVID) interferes in vivo with the B-cell receptor (BCR) signaling by increasing constitutive ERK activation and by reducing the phosphorylated ERK increment induced by BCR cross-linking. " 07/01/2014 - "A descriptive and prospective study was done including adult patients con definitive diagnosis of common variable immunodeficiency, receiving IVIg at replacement dose every 3 weeks. " 04/01/2014 - "Its routine use is recommended over other formulas in common variable immunodeficiency patients with suspected renal disease secondary to the use of IVIG. "
3.	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. " 01/01/2015 - "Surface Plasmon Resonance Analysis Shows an IgG-Isotype-Specific Defect in ABO Blood Group Antibody Formation in Patients with Common Variable Immunodeficiency." 01/01/2014 - "We also reviewed charts of consecutive adult index patients with primary Ig deficiency (35 common variable immunodeficiency, 406 IgG subclass deficiency) to identify other patients with probable ASHNL. " 01/01/2012 - "Management of a pregnant woman with common variable immunodeficiency and previous reactions to intravenous IgG administration." 07/08/2010 - "Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum immunoglobulin G, A, and/or M with loss of antibody production. "
4.	Bruton type agammaglobulinemiaIBA 01/01/2012 - "Recent studies have demonstrated that patients with common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) have altered TLR responsiveness. " 07/01/2015 - "Prospective evaluation of 18 patients with Common Variable Immunodeficiency and X-linked Agammaglobulinemia. " 12/01/2014 - "Fifty patients had common variable immunodeficiency (CVID), 11 had X-linked agammaglobulinemia (XLA), and 55 had other immune system disorders. " 01/01/2013 - "Gastrointestinal symptoms are common in patients with common variable immunodeficiency disorders (CVID) and less frequent in X-linked agammaglobulinemia (XLA) although the exact prevalence is not well established. " 01/01/2013 - "Outcome of screening endoscopy in common variable immunodeficiency disorder and X-linked agammaglobulinemia."
5.	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."
6.	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). " 11/01/2004 - "Assessing thymopoiesis in patients with common variable immunodeficiency as measured by T-cell receptor excision circles." 08/01/2002 - "Assessment of thymic output in common variable immunodeficiency patients by evaluation of T cell receptor excision circles." 12/01/2000 - "Characterization of the T cell receptor repertoire in patients with common variable immunodeficiency: oligoclonal expansion of CD8(+) T cells." 11/01/1997 - "Defective integration of activating signals derived from the T cell receptor (TCR) and costimulatory molecules in both CD4+ and CD8+ T lymphocytes of common variable immunodeficiency (CVID) patients."
7.	Cimetidine (Biomet)FDA LinkGeneric 11/01/1989 - "Common variable immunodeficiency: a family study and therapeutic trial with cimetidine." 01/01/1987 - "[Immunomodulator therapy with cimetidine in patients with common variable immunodeficiency]." 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. "
8.	gamma-Globulins (gamma-Globulin)IBA 07/01/2004 - "Common variable immunodeficiency syndrome (CVID) was suspected and diagnosis was established by demonstrating a significant reduction of plasma gamma-globulin levels. " 02/18/1995 - "[The use of human gamma globulin in the treatment of common variable immunodeficiency]." 03/01/1994 - "In this article, we describe the results of initiating a therapeutic regimen of high doses (400 mg/kg) of intravenous gamma globulin every 3 weeks starting in the first trimester of pregnancy for a woman with common variable immunodeficiency. " 09/01/1987 - "Because of evidence of immunoregulatory effects of short-term administration of pooled donor gamma globulins, the effects of intravenous immune serum globulin were studied in nine adult patients with common variable hypogammaglobulinemia over a two-year period. " 03/01/1994 - "Bioavailability of gamma-globulin after subcutaneous infusions in patients with common variable immunodeficiency."
9.	InterferonsIBA 09/01/1997 - "Sustained response to interferon in a patient with common variable immunodeficiency and hepatitis C." 01/01/1987 - "The production of interferon (IFN) after stimulation of peripheral blood mononuclear cells with Sendai virus or phytohemagglutinin was studied in patients with common variable immunodeficiency (CVID) or selective IgA deficiency. " 06/01/1986 - "Patients with Bruton's agammaglobulinemia demonstrated normal NK and interferon generation, as did patients with common variable immunodeficiency, even when subdivided into patients with T-cell proliferative deficiencies and those with only hypogammaglobulinemia. " 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." 07/01/2014 - "De novo autoimmune hepatitis after interferon treatment in a liver transplant recipient with common variable immunodeficiency."
10.	Prostaglandin-Endoperoxide Synthases (Cyclooxygenase)IBA 11/01/1991 - "Improved in vitro antigen-specific antibody synthesis in two patients with common variable immunodeficiency taking an oral cyclooxygenase and lipoxygenase inhibitor (ketoprofen)."

Therapies and Procedures

1.	Transplantation (Transplant Recipients) 07/01/2014 - "De novo autoimmune hepatitis after interferon treatment in a liver transplant recipient with common variable immunodeficiency." 01/01/2002 - "The therapeutic effect of pooled intravenous immunoglobulin on idiopathic late-onset immunoglobulin deficiency, Wegener's granulomatosis, serious infections and efficacy of immune globulin in preventing of cytomegalovirus disease in lung-transplant recipients was reported."
2.	Aftercare (After-Treatment) 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."
3.	Liver Transplantation 01/01/2014 - "Liver transplantation in patients with common variable immunodeficiency: a report of two cases." 12/01/2013 - "Acquired hypogammaglobulinemia in HIV-positive subjects after liver transplantation." 12/01/2013 - "Using serum samples from the Solid Organ Transplantation in HIV: Multi-Site Study, we studied the epidemiology of acquired hypogammaglobulinemia (HGG) after liver transplantation (LT) in 79 HIV-infected individuals with a median CD4 count at enrollment of 288 (interquartile range 200-423) cells/μL. " 10/01/2013 - "Aspergillosis after liver transplantation in the context of common variable immunodeficiency: case report." 09/01/2012 - "Liver transplantation for severe hepatitis in patients with common variable immunodeficiency."
4.	Immunomodulation 01/01/1996 - "The critical role of CD40-CD40 ligand-mediated effects in T cell-dependent B cell activation, shown through the study of patients with common variable immunodeficiency and hyper IgM syndrome, suggests a potential narrow target for immunomodulatory therapy. " 11/01/2015 - "A retrospective review of medical records at Mount Sinai Medical Center in New York was conducted for referred patients with common variable immunodeficiency, CT scan-confirmed interstitial lung disease, and periodic pulmonary function testing covering 20 or more months before immunomodulatory therapy. " 01/01/2006 - "We have identified a patient with common variable immunodeficiency (CVID)-associated sprue complicated by duodenal cytomegalovirus (CMV) infection following corticosteroid and ancillary immunomodulatory therapy. "
5.	Organ Transplantation 10/01/2011 - "Six patients were HIV+, whereas four had received organ transplantations, one was affected by common variable immunodeficiency and four had a recent history of malignancy. " 12/01/2013 - "Using serum samples from the Solid Organ Transplantation in HIV: Multi-Site Study, we studied the epidemiology of acquired hypogammaglobulinemia (HGG) after liver transplantation (LT) in 79 HIV-infected individuals with a median CD4 count at enrollment of 288 (interquartile range 200-423) cells/μL. "