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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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What is the Immune System?01:38

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Special Features of Adaptive Immunity01:20

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The adaptive immune system, a crucial component of the overall immune response, offers a highly specialized defense against pathogens. It involves specific cell types and features, enabling it to combat infections effectively and efficiently.
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
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Cells of the Adaptive Immune Response01:23

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The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...
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Introduction to Innate and Adaptive Immunity01:21

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The human immune system is a complex defense mechanism that protects the body from harmful pathogens and foreign substances. It comprises two crucial components: innate and adaptive immunity.
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Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
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Autoimmunity as a continuum in primary immunodeficiency.

Jolan E Walter1,2,3, Irmel A Ayala4, Diana Milojevic5

  • 1Division of Allergy/Immunology in Department of Pediatrics, University of South Florida, Tampa.

Current Opinion in Pediatrics
|November 7, 2019
PubMed
Summary
This summary is machine-generated.

Primary immunodeficiency disorders (PIDs) often present with autoimmunity, not just infections. Early diagnosis through genetic and immune studies enables targeted treatments and improves outcomes for these complex conditions.

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Primary immunodeficiency disorders (PIDs) are increasingly recognized to manifest with autoimmune conditions, expanding beyond their traditional definition centered on infections.
  • Autoimmunity, including cytopenias, arthritis, and enteropathy, can be the initial clinical sign or a sequela of PIDs.

Purpose of the Study:

  • To review the latest multidisciplinary approaches for understanding the expanding clinical phenotypes of PIDs associated with autoimmunity.
  • To discuss novel gene defects and presentations of known PID entities with autoimmune features.
  • To cover diagnostic tools, mechanistic insights, and therapeutic strategies, including hematopoietic stem-cell transplantation (HSCT).

Main Methods:

  • Review of recent literature on PIDs with autoimmunity.
  • Analysis of diagnostic advancements, including genetic screening, functional studies, and immunological biomarkers.
  • Examination of clinical data from large cohort studies detailing specific gene defects (e.g., CTLA4, LRBA, PI3Kδ, NFKB1, RAG).

Main Results:

  • Increased awareness and advanced diagnostic tools facilitate earlier recognition of PIDs in patients with autoimmunity.
  • Large cohort studies have elucidated the clinical spectrum and treatment outcomes for PIDs linked to specific genetic defects.
  • Early identification allows for targeted therapies, immune pathway re-balancing with biologics, or definitive treatment with HSCT.

Conclusions:

  • The clinical presentation of PIDs with autoimmunity is highly variable.
  • In-depth diagnostics and precision medicine approaches are crucial for managing these complex cases.
  • Early and accurate diagnosis is key to initiating effective, targeted treatments and improving patient outcomes.