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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

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
The immune system...
Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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 disorders...
Type I Diabetes I: Introduction01:12

Type I Diabetes I: Introduction

Type 1 diabetes mellitus is a chronic metabolic disorder characterized by an absolute deficiency of insulin resulting from the autoimmune destruction of pancreatic β-cells. Although it can occur at any age, it is most commonly diagnosed in childhood, adolescence, or early adulthood. The loss of insulin production impairs cellular glucose uptake, resulting in persistent hyperglycemia and necessitating lifelong insulin therapy.Autoimmune Destruction of β-CellsThe hallmark of type 1 diabetes is an...
Type I Diabetes II: Pathophysiology01:26

Type I Diabetes II: Pathophysiology

Type 1 diabetes mellitus arises from an immune-mediated destruction of pancreatic β-cells, resulting in an absolute deficiency of insulin. This process develops in genetically susceptible individuals when autoimmunity, environmental exposures, and immunologic dysregulation converge to trigger a targeted attack on the insulin-producing cells of the pancreas. The β-cells are located within the islets of Langerhans and are essential for regulating blood glucose by facilitating cellular uptake of...
What is the Immune System?01:38

What is the Immune System?

Overview
Inflammatory Bowel Disease III: Crohn's Disease01:25

Inflammatory Bowel Disease III: Crohn's Disease

Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...

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Related Experiment Video

Updated: May 20, 2026

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
11:12

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity

Published on: April 11, 2019

Pathogenesis of autoimmunity in common variable immunodeficiency.

Klaus Warnatz1, Reinhard E Voll

  • 1Centre of Chronic Immunodeficiency, University Medical Center Freiburg, University of Freiburg, Freiburg, Germany.

Frontiers in Immunology
|July 25, 2012
PubMed
Summary

Common variable immunodeficiency (CVID) frequently involves autoimmune (AI) issues, particularly AI cytopenias affecting blood cells. Understanding these AI manifestations in CVID offers insights into broader autoimmunity mechanisms.

Keywords:
autoimmunityCD21low B cellsautoimmune cytopeniacommon variable immunodeficiencyhypogammaglobulinemia

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Published on: September 26, 2013

Area of Science:

  • Immunology
  • Genetics
  • Autoimmunity

Background:

  • Common variable immunodeficiency (CVID) is frequently associated with autoimmune (AI) manifestations, affecting up to 25% of patients.
  • AI dysregulation appears integral to CVID, often presenting early and involving antibody-mediated AI cytopenias (erythrocytes, platelets) in over 50% of cases.
  • This contrasts with cell-mediated organ-specific autoimmunity and may resemble AI lymphoproliferative syndrome in some patients.

Purpose of the Study:

  • To explore the relationship between autoimmune manifestations and CVID.
  • To investigate the immunological and genetic factors contributing to autoimmunity in CVID patients.
  • To understand the general mechanisms of human autoimmunity through CVID research.

Main Methods:

  • Analysis of patient cohorts with CVID and autoimmune manifestations.
  • Immunological examination of B and T cell homeostasis in affected individuals.
  • Investigation of genetic defects (e.g., TACI, CD19, CD81) and serum biomarkers (e.g., BAFF) associated with CVID and autoimmunity.

Main Results:

  • The majority of CVID patients with AI cytopenias exhibit dysregulated B and T cell homeostasis.
  • Altered antigen receptor signaling and elevated B cell-activating factor (BAFF) serum levels may contribute to autoimmunity in CVID.
  • Specific genetic defects, including alterations in TACI, CD19, and CD81, are frequently linked to AI manifestations in CVID.

Conclusions:

  • Autoimmunity is a significant feature of CVID, distinct from other autoimmune conditions.
  • Dysregulated immune cell homeostasis and specific genetic factors play crucial roles in CVID-associated autoimmunity.
  • Studying autoimmunity in CVID provides valuable insights into the fundamental mechanisms underlying human autoimmunity.