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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.
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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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A Rapid, Simple, and Standardized Homogenization Method to Prepare Antigen/Adjuvant Emulsions for Inducing Experimental Autoimmune Encephalomyelitis
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[AUTOIMMUNITY AND IMMUNODEFICIENCIES].

Alenka Gagro

    Reumatizam
    |April 7, 2018
    PubMed
    Summary
    This summary is machine-generated.

    Primary immunodeficiencies, often linked to immune dysregulation, can paradoxically cause autoimmune diseases. Understanding these genetic defects is key to explaining the overlap between immunodeficiency and autoimmunity.

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    Area of Science:

    • Immunology
    • Genetics
    • Rheumatology

    Background:

    • Autoimmune diseases and primary immunodeficiencies share immune dysregulation pathways.
    • While typically polygenic, autoimmune diseases can arise from single-gene defects in immune components.
    • This highlights a significant overlap between immunodeficiency and autoimmunity.

    Purpose of the Study:

    • To review the incidence, clinical presentation, and mechanisms of autoimmunity in primary immunodeficiency patients.
    • To provide insights relevant to rheumatologists managing these complex cases.

    Main Methods:

    • This is a clinically-oriented review.
    • It synthesizes current knowledge on monogenic defects leading to autoimmunity.

    Main Results:

    • Single gene defects can lead to autoimmunity, though mechanisms are not fully elucidated.
    • Primary immunodeficiencies present with autoimmune manifestations.

    Conclusions:

    • Understanding monogenic defects offers new insights into autoimmune disease development.
    • Rheumatologists should consider primary immunodeficiencies in patients with unexplained autoimmunity.