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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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What is the Immune System?01:38

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Autoimmune Disorders01:29

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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.
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The immune...
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Humoral Immune Responses01:36

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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.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
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Related Experiment Video

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

Amrita Dosanjh1

  • 1Department of Pediatrics, Rady Childrens Hospital, San Diego, CA.

Pediatrics in Review
|November 4, 2015
PubMed
Summary

Primary immunodeficiencies, like immunoglobulin A deficiency, are linked to autoimmune diseases. These conditions can affect mucosal immunity, leading to issues such as arthritis and anemia.

Area of Science:

  • Immunology
  • Genetics
  • Rheumatology

Background:

  • Primary immunodeficiencies (PIDs) are a group of rare genetic disorders affecting the immune system.
  • A significant subset of PIDs is associated with the development of autoimmune diseases.
  • Understanding these associations is crucial for diagnosis and management.

Purpose of the Study:

  • To review the association between primary immunodeficiencies and autoimmune diseases.
  • To highlight common PIDs linked to autoimmunity based on available evidence.
  • To discuss the immunological mechanisms underlying these associations.

Main Methods:

  • Review of existing literature, including level A evidence studies (randomized controlled trials, meta-analyses).
  • Inclusion of level B evidence studies (cohort studies, case-control studies).

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  • Consideration of illustrative case reports (level D evidence) for rare conditions.

    • Main Results:

    • Immunoglobulin A deficiency is the most common PID, linked to defective mucosal immunity and autoimmunity.
    • Wiskott Aldrich syndrome, presenting early, is associated with autoimmune arthritis and anemia.
    • Several PIDs involve defects in T regulatory cells and cytokine signaling, leading to autoantibody production.
    • Complement deficiencies are associated with autoimmune diseases, particularly systemic lupus erythematosus.

    Conclusions:

    • Primary immunodeficiencies represent a significant risk factor for developing autoimmune diseases.
    • Defects in immune regulation, including T regulatory cells and complement pathways, are key mechanisms.
    • Early identification and management of PIDs are essential to mitigate autoimmune complications.