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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency...
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Autoimmune Disorders01:29

Autoimmune Disorders

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Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
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Skin Diseases and Disorders01:23

Skin Diseases and Disorders

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Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
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Antigens Involved in Adaptive Immunity01:26

Antigens Involved in Adaptive Immunity

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An antigen is any substance the immune system identifies as foreign and potentially harmful to the body, prompting an immune response. Antigens have two functional properties: immunogenicity and reactivity. Immunogenicity is the ability of an antigen to stimulate a specific immune response. At the same time, reactivity describes the antigen's ability to react with the cells and antibodies produced in response to it.
Complete Antigens
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T Cell Types and Functions01:24

T Cell Types and Functions

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When T cells with CD4 markers are activated, they give rise to two types of effector cells: helper T cells and regulatory T cells. Meanwhile, T cells with CD8 markers differentiate into effector cytotoxic T cells. The differentiation of CD4 T cells into helper T cell subsets, such as Th1, Th2, and Th17 cells, is dependent on the antigen type, antigen-presenting cell, and regulatory cytokines.
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
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What is the Immune System?01:38

What is the Immune System?

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Overview
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Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry
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[Autoimmune diseases in patients with common variable immunodeficiency].

Laura Berrón-Ruiz1

  • 1Instituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias, Ciudad de México, México. lberronruiz@yahoo.com.mx.

Revista Alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)
|June 21, 2021
PubMed
Summary

Common variable immunodeficiency (CVID) involves low immunoglobulin levels and recurrent infections. Autoimmunity affects 30% of CVID patients, presenting a major clinical challenge with genetic insights improving understanding.

Keywords:
AutoimmunityCommon variable immunodeficiencyPrimary immunodeficiency

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency affecting 1 in 10,000–50,000 people.
  • CVID presents with low immunoglobulin levels, poor antibody response, and recurrent bacterial infections.
  • A significant subset of CVID patients exhibits immune deregulation, including autoimmunity.

Purpose of the Study:

  • To explore the link between CVID and autoimmune diseases.
  • To highlight the role of genetic defects in CVID-associated autoimmunity.
  • To emphasize the diagnostic and understanding advancements in CVID immune deregulation.

Main Methods:

  • Review of existing literature on CVID and autoimmunity.
  • Analysis of monogenic defects contributing to CVID pathogenesis.
  • Discussion of new diagnostic tools, particularly genetic testing.

Main Results:

  • Approximately 30% of CVID patients develop autoimmune conditions.
  • Autoimmune complications include autoimmune cytopenia and organ-specific diseases like IBD, celiac disease, and lung disease.
  • Monogenic defects are elucidating the mechanisms behind combined immunodeficiency and autoimmunity.

Conclusions:

  • Autoimmune diseases represent a major clinical challenge in CVID management.
  • Genetic discoveries are crucial for understanding the immunopathology of CVID with autoimmunity.
  • Advanced diagnostic tools, especially genetic ones, enhance the comprehension of immune deregulation in CVID.