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Review
. 2019 Nov;123(5):454-460.
doi: 10.1016/j.anai.2019.07.014. Epub 2019 Jul 23.

Autoimmunity in common variable immunodeficiency

Shradha Agarwal  1 , Charlotte Cunningham-Rundles  2
Affiliations
Review

Autoimmunity in common variable immunodeficiency

Shradha Agarwal et al. Ann Allergy Asthma Immunol. 2019 Nov.

Abstract

Objective: Common variable immunodeficiency (CVID) is a primary immunodeficiency that is clinically heterogeneous, characterized by both infectious and noninfectious complications. Although the hallmark of disease presentation is commonly a history of recurrent sinopulmonary infections, autoimmunity and noninfectious inflammatory conditions are increasingly associated with CVID.

Data sources: A comprehensive literature search using PubMed of basic science and clinical articles was performed.

Study selections: Articles discussing the association of autoimmunity with primary immunodeficiency, specifically CVID, were selected.

Results: The most common autoimmune conditions are cytopenias, including immune thrombocytopenia purpura and hemolytic anemia, but organ-specific autoimmune/inflammatory complications involving the gastrointestinal, skin, joints, connective tissue, and respiratory tract. In most cases, immunoglobulin replacement therapy does not ameliorate or treat these inflammatory complications, and additional immunomodulatory treatments are needed.

Conclusion: Mechanisms producing these conditions are poorly understood but include cytokine and cellular inflammatory pathways, and loss of tolerance to self-antigens through the multiple signaling molecules and pathways common to tolerance and immune deficiency.

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Figures

Figure 1.
Figure 1.
Inflammatory or autoimmune manifestations of New York CVID cohort. Resnick et al. Percentage of patients (n = 473) with infections only, or others who had additional complications.
Figure 2.
Figure 2.
Autoimmunity in CVID cohort of USIDNET. Feuille et al. Autoimmune conditions (n = 351) in 990 CVID patients in USIDNET.
See this image and copyright information in PMC

Comment in

  • Captaining the PIDD ship.
    Wasserman RL. Wasserman RL. Ann Allergy Asthma Immunol. 2019 Nov;123(5):419. doi: 10.1016/j.anai.2019.09.013. Ann Allergy Asthma Immunol. 2019. PMID: 31676017 No abstract available.

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