Autoimmunity in Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is a primary immunodeficiency that is clinically heterogeneous, characterized by both infectious and non-infectious complications. While the hallmark of disease presentation is commonly a history of recurrent sinopulmonary infections, autoimmunity and non-infectious inflammatory conditions are increasing associated with CVID.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Review Source Type: research

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Since the early identification of specific pathogenic variants in genes encoding Bruton ’s tyrosine kinase (BTK), Wiskott Aldrich syndrome protein (WASp) and the gamma chain of the interlukin-2 receptor (IL2RG), the evolution of DNA sequencing technologies has led to the identification of variants in over 350 genes causing Primary Immune Deficiency Disorders (PIDD), with new discover ies being reported each year.1 Prior to the widespread availability of genetic testing, diagnosis of PIDD relied upon assays that provide evidence of immune dysfunction, but are not typically themselves diagnostic and have various limitations.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Source Type: research
PMID: 31472268 [PubMed - as supplied by publisher]
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Ann Allergy Asthma Immunol Source Type: research
A significant proportion of primary immunodeficiency (PID) diseases include humoral deficiency. Humoral deficiencies range in severity from complete deficiencies observed in diseases such as X-linked or autosomal recessive agammaglobulinemias and certain severe combined immune deficiencies (SCID), to milder specific antibody deficiencies. According to a recent update from the Jeffrey Modell Centers Network, disorders classified as primarily humoral deficiencies accounted for 45% of survey center PID patients worldwide, not including the many additional patients with combined immune deficiencies who also suffer from antibody deficiency.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: CME Reviews Source Type: research
Blancas-Galicia L Abstract Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defective phagocytic NADPH oxidase, causing a complete lack or significant decrease in the production of microbicidal reactive oxygen metabolites. It mainly affects male children; however, there are scarce reports of adult females diagnosed with X-linked-CGD attributed to an extremely skewed X-chromosome inactivation. This condition is characterized by severe and recurrent infections that usually develop after childhood. In clinical practice, physicians who usually confront these patients should suspect this enti...
Source: Iranian Journal of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Iran J Allergy Asthma Immunol Source Type: research
Severe combined immune deficiency (SCID) is the first primary immunodeficiency amenable to population-based newborn screening (NBS) and is now available in all 50 states and Puerto Rico. NBS for SCID uses real-time quantitative polymerase chain reaction to measure the number of copies of T-cell restriction excision circles (TRECs) in DNA from newborn dried blood spots and reliably detects severe T-cell lymphopenia (TCL) at birth. SCID is the primary condition detected using this assay. However, it is well known that many secondary conditions, with varying degrees of TCL, are identified through SCID NBS.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Perspective Source Type: research
Severe Combined Immune Deficiency (SCID) is the first primary immunodeficiency amenable to population based newborn screening (NBS) and is now available in all 50 states and Puerto Rico. NBS for SCID uses real time qPCR to measure the number of copies of T cell restriction excision circles (TREC) in DNA from newborn dried blood spots (DBS) and reliably detects severe T cell lymphopenia (TCL) at birth. SCID is the primary condition detected using this assay. However, it is well known that many secondary conditions, having varying degrees of TCL, are identified through SCID NBS.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Perspective Source Type: research
As implied by its name, common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency in adults and is a diagnosis frequently considered by the practicing allergist and clinical immunologist. Despite familiarity, there is disagreement about which immune deficient patients should be given the diagnosis of CVID. As the name also implies, patients with CVID often exhibit protean clinical manifestations1, 2. Reflecting this, the International Union of Immunological Societies (IUIS) Classification of Primary Immunodeficiency refers to CVID as a group of disorders with several clinical and la...
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Review Source Type: research
CONCLUSION: Mechanisms producing these conditions are poorly understood but include cytokine and cellular inflammatory pathways, and loss of tolerance to self-antigens through the multiple signaling molecules and pathways common to tolerance and immune deficiency. PMID: 31349011 [PubMed - as supplied by publisher]
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Ann Allergy Asthma Immunol Source Type: research
Abstract BACKGROUND: The subcutaneous immune globulin (SCIG) 20% product, Ig20Gly, was shown to be efficacious and well tolerated in two phase 2/3 North American and European studies at infusion volumes up to 60 mL/site and rates up to 60 mL/h/site in patients with primary immunodeficiency diseases. OBJECTIVE: To assess patient experience after switching to Ig20Gly with fast infusion rates and large infusion volumes/site in the North American study. METHODS: In this analysis of the open-label phase 2/3 study in which patients aged ≥2 years received weekly Ig20Gly infusions for up to approximately 1.3 ...
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Ann Allergy Asthma Immunol Source Type: research
The subcutaneous immune globulin (SCIG) 20% product, Ig20Gly, was shown to be efficacious and well tolerated in two phase 2/3 North American and European studies at infusion volumes up to 60 mL/site and rates up to 60 mL/h/site in patients with primary immunodeficiency diseases.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Source Type: research
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