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Immunodeficiency Diseases

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.
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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
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Factors Affecting the Risk of Infection

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The hosts' susceptibility to infection depends on several factors. The integrity of the skin and mucous membranes helps protect the body against microbial attacks. When the skin is altered, the chance of infection, limb loss, and even death increases.
The integrity and count of the white blood cells help the body resist pathogens and fight infection. When impaired, it reduces the body's resistance to pathogens. The acidic pH levels of the gastrointestinal, genitourinary tracts, and skin...

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Unbalanced Immune System: Immunodeficiencies and Autoimmunity.

Giuliana Giardino¹, Vera Gallo¹, Rosaria Prencipe¹

¹Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.

Frontiers in Pediatrics

|October 22, 2016

Summary

Primary immunodeficiencies (PIDs) increase the risk of autoimmune diseases due to immune dysregulation. This review details autoimmune manifestations and their underlying mechanisms in various PIDs.

Keywords:

autoimmune hemolytic anemia autoimmunity immune thrombocytopenia immunodeficiency systemic lupus erytematous

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Area of Science:

Immunology
Autoimmunity
Genetics

Background:

Primary immunodeficiencies (PIDs) are associated with an increased risk of autoimmune manifestations.
Autoimmunity and immune deficiency are intertwined, reflecting inadequate immune function in PIDs.
Mechanisms include defects in self-antigen tolerance and chronic antigen stimulation.

Approach:

This review summarizes autoimmune manifestations across different PIDs.
It includes humoral immunodeficiencies, combined immunodeficiencies, and syndromes with immunodeficiencies.
Pathogenetic mechanisms underlying autoimmunity in specific PIDs are explained where possible.

Key Points:

Immune dysregulation in PIDs leads to exaggerated inflammatory responses.
Compensatory inflammation can result in tissue damage and autoimmunity.
Distinct PIDs exhibit unique autoimmune manifestations and pathogenetic mechanisms.

Conclusions:

Autoimmunity is a significant concern in various primary immunodeficiencies.
Understanding the specific mechanisms is crucial for managing autoimmune complications in PIDs.
This review provides a comprehensive overview of autoimmunity in PIDs.