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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.

Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology
|July 27, 2019
PubMed
Summary
This summary is machine-generated.

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.

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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.