Autoimmunity in common variable immunodeficiency
Shradha Agarwal1, Charlotte Cunningham-Rundles1
1Icahn School of Medicine at Mount Sinai, Division of Allergy and Clinical Immunology, Department of Medicine, New York, New York.
Summary
Common variable immunodeficiency (CVID) increases the risk of autoimmune conditions like cytopenias and organ-specific inflammation. These complications often require immunomodulatory treatments beyond immunoglobulin replacement therapy.
Area of Science:
- Immunology
- Clinical Medicine
Background:
- Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency.
- While recurrent sinopulmonary infections are typical, autoimmune and inflammatory conditions are increasingly recognized CVID complications.
Purpose of the Study:
- To review the association between autoimmunity and Common variable immunodeficiency (CVID).
Main Methods:
- A comprehensive literature search of basic science and clinical articles was conducted using PubMed.
- Articles specifically addressing autoimmunity in CVID were selected for review.
Main Results:
- Autoimmune complications in CVID are diverse, including cytopenias (e.g., immune thrombocytopenia, hemolytic anemia) and organ-specific inflammation affecting the GI tract, skin, joints, connective tissues, and respiratory system.
- Immunoglobulin replacement therapy is often insufficient for managing these inflammatory issues, necessitating additional immunomodulatory treatments.
Conclusions:
- The underlying mechanisms for CVID-associated autoimmunity are not fully understood.
- These mechanisms likely involve cytokine and cellular inflammatory pathways, as well as a breakdown in self-tolerance due to shared signaling pathways in immune deficiency.
Related Concept Videos
01:25Immunodeficiency Diseases
2.0K
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...
There are three main causes of immunodeficiency...
2.0K
03:24Common Ion Effect
46.0K
Compared with pure water, the solubility of an ionic compound is less in aqueous solutions containing a common ion (one also produced by dissolution of the ionic compound). This is an example of a phenomenon known as the common ion effect, which is a consequence of the law of mass action that may be explained using Le Châtelier’s principle. Consider the dissolution of silver iodide:
46.0K
01:26Variables Affecting Phosphorescence and Fluorescence
1.3K
Fluorescence and phosphorescence are essential phenomena in fields like analytical chemistry, biological imaging, and materials science, where they detect molecular properties and visualize cellular structures. Understanding the variables that influence these luminescent behaviors is crucial for maximizing accuracy and efficiency in their applications. These variables can broadly be grouped into chemical structure, solvent properties, and external conditions, each playing a distinct role in...
1.3K
01:11Variability: Analysis
450
Measures of variability are statistical metrics that reveal the dispersion pattern within a dataset. They are pivotal in biostatistics, providing insights into the heterogeneity within health and biological data. Variability signifies the degree to which data points diverge from one another, helping researchers understand the potential range of values and associated uncertainty within the data.
The range is a simple measure of variability, indicating the difference between the highest and...
The range is a simple measure of variability, indicating the difference between the highest and...
450
01:09Random Variables
17.5K
A random variable is a single numerical value that indicates the outcome of a procedure. The concept of random variables is fundamental to the probability theory and was introduced by a Russian mathematician, Pafnuty Chebyshev, in the mid-nineteenth century.
Uppercase letters such as X or Y denote a random variable. Lowercase letters like x or y denote the value of a random variable. If X is a random variable, then X is written in words, and x is given as a number.
For example, let X = the...
Uppercase letters such as X or Y denote a random variable. Lowercase letters like x or y denote the value of a random variable. If X is a random variable, then X is written in words, and x is given as a number.
For example, let X = the...
17.5K
01:29Autoimmune Disorders
1.5K
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...
Concept and Mechanism of Autoimmune Diseases
The immune...
1.5K