TY - JOUR
T1 - Autoimmunity in common variable immunodeficiency
AU - Agarwal, Shradha
AU - Cunningham-Rundles, Charlotte
N1 - Publisher Copyright:
© 2019 American College of Allergy, Asthma & Immunology
PY - 2019/11
Y1 - 2019/11
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85070928358&partnerID=8YFLogxK
U2 - 10.1016/j.anai.2019.07.014
DO - 10.1016/j.anai.2019.07.014
M3 - Review article
C2 - 31349011
AN - SCOPUS:85070928358
SN - 1081-1206
VL - 123
SP - 454
EP - 460
JO - Annals of Allergy, Asthma and Immunology
JF - Annals of Allergy, Asthma and Immunology
IS - 5
ER -