Abstract:
:Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency among adults and is characterized by a B cell dysfunction and increased risk of respiratory tract infections with encapsulated bacteria. However, a large proportion of patients also has inflammatory and autoimmune complications. It may seem like a paradox that immunodeficiency and inflammation/autoimmunity coexist within the same individuals. In this commentary, we propose that CVID immunopathogenesis involves an interplay of genes, environmental factors, and dysregulation of immune cells, where gut microbiota and gastrointestinal inflammation can both be important contributors or endpoints to the systemic immune activation seen in CVID, and where epigenetic mechanism may be the undiscovered link between these contributors. In our opinion, these pathways could represent novel targets for therapy in CVID directed against autoimmune and inflammatory manifestations that represent the most severe complications in these patients. Considering the heterogeneous nature of CVID, these mechanisms may not be present in all patients, and different complications may be triggered by different risk factors. CVID is really a variable disease and in the future there is clearly a need for a more personalized medicine based on both genotypic and phenotypic findings.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Jørgensen SF,Fevang B,Aukrust Pdoi
10.1007/s10875-018-0574-zsubject
Has Abstractpub_date
2019-01-01 00:00:00pages
30-36issue
1eissn
0271-9142issn
1573-2592pii
10.1007/s10875-018-0574-zjournal_volume
39pub_type
杂志文章,评审abstract::Five common variable immunodeficiency (CVI) patients were analyzed for expression of immunoglobulin (Ig) genes. In the pokeweed mitogen (PWM)-induced Ig-production assay, the combination of T and B cells showed that all patients' T cells had normal helper functions and all patients' B cells had profound defects. The d...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918184
更新日期:1991-09-01 00:00:00
abstract::Allogeneic bone marrow-engrafted adults immunized with meningococcal types A and C and pneumococcal type 14 polysaccharide antigens showed only low antibody titers of the IgM class, no antibody titers of the IgG or IgA classes, and no bactericidal activity in vitro. The analytical isoelectrofocusing showed the appeara...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917916
更新日期:1990-05-01 00:00:00
abstract::We read with interest the recent paper in the JoCI, entitled 'Study of the expression of Toll-Like Receptors in Different Histological Types of Colorectal Polyps and Their Relationship with Colorectal Cancer' by Eiró et al. TLR7 and 9 expression is altered in CRC vs. normal control; which is proposed to be correlated ...
journal_title:Journal of clinical immunology
pub_type: 评论,信件
doi:10.1007/s10875-012-9719-7
更新日期:2012-12-01 00:00:00
abstract::These studies report findings that demonstrate that human alpha lymphotoxin (LT) induces local, visible, and microscopic inflammatory reactions in normal skin. Skin sites in rabbits, when inoculated with a single injection of native or recombinant human alpha lymphotoxin, demonstrated erythema, swelling, and warmth wi...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915556
更新日期:1987-07-01 00:00:00
abstract::Serum amyloid P component (SAP) binds in vitro to DNA; based on findings in SAP-deficient mice it was proposed that SAP's role is to handle chromatin and DNA, thereby preventing formation of anti-DNA antibodies. For the first time we have shown the presence of Ca2+-dependent SAP-DNA complexes, measured by ELISA, in se...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1026478914129
更新日期:2000-11-01 00:00:00
abstract:INTRODUCTION:This study was done to further reveal the role of the innate immune system in celiac disease. METHODS:Dendritic cells were matured from venous blood of patients with active or treated celiac disease and DQ2-DQ8-positive or negative controls. Dendritic cells were treated with a peptic-tryptic digest of gli...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-008-9228-x
更新日期:2009-01-01 00:00:00
abstract::The present study analyzes the role of CD28-B7-mediated costimulation during in vitro human peripheral blood memory T cell activation by influenza A virus. Inhibition studies using the B7-binding fusion protein CTLA4Ig and antibodies against CD80 and CD86 demonstrate that CTLA4Ig and anti-CD86 inhibited influenza-spec...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1010987426835
更新日期:2001-07-01 00:00:00
abstract::We studied immunological functions of peripheral blood lymphocytes (PBL) from human T-cell leukemia virus type I (HTLV-I)-seropositive healthy carriers in vitro. Proliferative responses of PBL to T-cell and B-cell mitogens such as concanavalin A (Con A), pokeweed mitogen (PWM), and Staphylococcus aureus Cowan I (SAC) ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918017
更新日期:1989-11-01 00:00:00
abstract::Atopic dermatitis in humans and dogs is a chronic relapsing allergic skin disease. Dogs show a spontaneous disease similar to the human counterpart and represent a model to improve our understanding of the immunological mechanisms, the pathogenesis of the disease, and new therapy development. The aim of the study was ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-010-9447-9
更新日期:2010-11-01 00:00:00
abstract:PURPOSE:Spondyloenchondrodysplasia is a rare immuno-osseous dysplasia caused by biallelic mutations in ACP5. We aimed to provide a survey of the skeletal, neurological and immune manifestations of this disease in a cohort of molecularly confirmed cases. METHODS:We compiled clinical, genetic and serological data from a...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-016-0252-y
更新日期:2016-04-01 00:00:00
abstract::CFSE dye dilution analysis and [3H] thymidine incorporation were used side by side to assess proliferative responses of peripheral blood mononuclear cells (PBMCs) after vaccination of renal cell carcinoma patients (n=6) with antigen-loaded dendritic cells. Immune responses against the control antigen keyhole limpet he...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章
doi:10.1007/s10875-004-6237-2
更新日期:2004-11-01 00:00:00
abstract:OBJECTIVES:The study clarified whether the T-helper (Th)1/Th2 imbalance existed only in coronary arterial inflammation or in both coronary arterial inflammation and myocardial inflammation and explored the significance of the imbalance of Th1/Th2 function after acute myocardial infarction (AMI). BACKGROUND:There are t...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-005-4088-0
更新日期:2005-05-01 00:00:00
abstract::The staphylococcal enterotoxins have been termed superantigens based on their ability to stimulate polyclonal proliferative responses of murine and human T lymphocytes expressing particular T-cell receptor V beta gene products. Certain of these toxins have been shown both to activate and to induce anergy in reactive T...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01540892
更新日期:1995-11-01 00:00:00
abstract::Cytokines are powerful mediator and communication molecules capable of regulating a wide spectrum of biologic functions, including immune responses. Although the role of cytokines in the pathogenesis of human disease is not yet understood, assays for cytokines have become a common feature in research and clinical labo...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/BF01546317
更新日期:1994-11-01 00:00:00
abstract:INTRODUCTION:Several differences have been described between neonatal and adult immune responses. The predisposition in early life to Th2-type response or tolerance makes it a susceptible period for infections and allergic sensitization. OBJECTIVE:The aim of this work was to evaluate the effects of CpG-containing olig...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9358-9
更新日期:2010-03-01 00:00:00
abstract::Rat T lymphoblasts arrested in the G1 phase of the cell cycle by interleukin-2 (IL-2) deprivation can be forced to proceed to the S phase when they are stimulated with IL-2 or the phorbol ester phorbol 12,13-dibutyrate (PDBu). When PDBu is used as a stimulus, extracellular regulated kinase 2 (ERK2) is activated by thr...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027375828134
更新日期:1997-11-01 00:00:00
abstract:BACKGROUND:Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase t...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-014-0001-z
更新日期:2014-05-01 00:00:00
abstract::The expression of chemokine receptors on leukocytes is related to their activation state. However, the exact mechanism underlying the induction of each chemokine receptor is poorly understood. Here, we investigated how CCR5, a chemokine receptor implicated in T cell trafficking and HIV infection, is induced in human T...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1011059906777
更新日期:2001-03-01 00:00:00
abstract::Intravenous immunoglobulin (IVIg) has been used to treat autoimmune diseases and lymphoid malignancies with some therapeutic effect. In both these pathological conditions, there is an overproduction of BAFF (for "B-cell-activating factor of the TNF family"), and APRIL (for "a proliferation-inducing ligand"). The prese...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9082-2
更新日期:2007-05-01 00:00:00
abstract:PURPOSE:Chronic granulomatous disease (CGD) is a primary immunodeficiency for which allogeneic hematopoietic stem cell transplant (HSCT) offers potential cure. Direct comparison of HSCT to non-HSCT management in the North American population was performed to identify clinical factors associated with overall survival (O...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-019-00635-2
更新日期:2019-05-01 00:00:00
abstract::Cross-sectional and limited sequential studies of neutrophil and monocyte chemotaxis were done using cells isolated from cord blood and peripheral blood of 2- to 6-day-old neonates and infants at 4 and 6 months of age. These studies show that cord blood phagocyte chemotaxis is comparable to adult values, whereas phago...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915228
更新日期:1982-07-01 00:00:00
abstract::To investigate the associations of IkappaBalpha gene polymorphisms with the development and clinical manifestations of systemic lupus erythematosus (SLE), 110 patients with SLE and 120 unrelated healthy controls were enrolled in this study. The IkappaBalpha -881 A/G, -826 C/T, -550 A/T, -519 C/T, and -297 C/T polymorp...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9156-1
更新日期:2008-05-01 00:00:00
abstract::MECP2 (methyl CpG binding protein 2) duplication causes syndromic intellectual disability. Patients often suffer from life-threatening infections, suggesting an additional immunodeficiency. We describe for the first time the detailed infectious and immunological phenotype of MECP2 duplication syndrome. 17/27 analyzed ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-015-0129-5
更新日期:2015-02-01 00:00:00
abstract::Increased levels of serum IgD can be found in single patients with a variety of clinical syndromes and in the disease entity designated hyper-IgD syndrome which is associated with periodic fever and lymphadenopathy. We investigated 17 patients, both children and adults, with high serum IgD levels ranging from 220 to 5...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/BF00917104
更新日期:1989-09-01 00:00:00
abstract:BACKGROUND:Nonalcoholic steatohepatitis (NASH) is a chronic inflammatory liver disease influenced by risk factors for the metabolic syndrome. In adult patients, NASH is associated with an altered phenotype and functionality of peripheral immune cells, the recruitment of leukocytes and intrahepatic activation, and an ex...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9635-2
更新日期:2012-06-01 00:00:00
abstract::The Bcl-2 family of proteins are important regulators of cell death. They are comprised of two opposing factions, the proapoptotic versus the antiapoptotic members. Both are required for normal development and cellular homeostasis of the immune system and other tissues. However, in certain circumstances they may parti...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1023/a:1020598632068
更新日期:1999-11-01 00:00:00
abstract::Chronic Granulomatous Disease (CGD), caused by genetic defects in components of the phagocyte NADPH oxidase pathway, leads to recurrent life-threatening bacterial and invasive fungal infections. While a number of unique pathogens have been associated with this disease, the causative organisms may be difficult to ident...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-013-9967-1
更新日期:2014-02-01 00:00:00
abstract:BACKGROUND:Several autoimmune disorders have been linked to adverse pregnancy outcome. IgA deficiency shares many autoimmune traits, but its association with pregnancy outcome is unknown. METHODS:Prospective population-based cohort study in Sweden of 613 mothers with IgA deficiency (IgA levels < .07 g/L) diagnosed in ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-014-0069-5
更新日期:2014-10-01 00:00:00
abstract::To investigate type II collagen (CII)-specific CD4+ T cell receptors involving in Collagen-induced arthritis (CIA) in DBA/1J mice as a model of rheumatoid arthritis in humans, TCR Vbeta usage in draining lymph nodes (dLNs) was assessed by flow cytometric analysis at 3, 5, and 8 weeks after bovine CII immunizations. In...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-006-9012-8
更新日期:2006-05-01 00:00:00
abstract::Various factors seem to be etiologic in the susceptibility to sinopulmonary infections in ataxia-telangiectasia (A-T) patients, i.e., low serum and salivary IgA, low serum IgG2, and even aspiration of saliva. S. pneumoniae is a common pathogen responsible from pulmonary infections and impaired antibody response to pol...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1020599810261
更新日期:1999-09-01 00:00:00