Clinical and Immunological Features of 78 Adult Patients with Primary Selective IgG Subclass Deficiencies
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The purpose of this study is to describe both clinical and immunological features in large cohort of adult patients with IgG subclass deficiency, and response to immunoglobulin therapy. This is a retrospective study of data obtained from electronic medical records and paper charts of 78 patients with IgG subclass deficiency seen and followed at our immunology clinics from 2010 to 2016. Both isolated selective IgG subclass deficiency as well as combined (two) subclass deficiencies were observed. IgG3 subclass deficiency, isolated and in combination with other IgG subclass deficiency, is the most frequent of IgG subclass deficiency. A majority of patients presented with upper and lower respiratory tract infections, especially chronic sinusitis. Both allergic and autoimmune manifestations are common; however, there is no subclass preference. The proportions and absolute numbers of CD3+ T cells, CD4+ T and CD8+ T cells, CD19+ B cells, and CD3−CD16+CD56+ NK cells were normal in the majority of patients in all IgG subclass deficiencies. Total serum IgG levels did not correlate with IgG subclass levels across all IgG subclass deficiencies. Anti-pneumococcal polysaccharide antibody responses were impaired in 56% of patients. IgG3 subclass deficiency is the most common IgG subclass deficiency, and anti-polysaccharide antibody responses are distributed among IgG subclasses with modest preference in IgG2 subclass. The majority of patients treated with immunoglobulin responded by reduction in frequency of infections and requirement of antibiotics.
KeywordsIgG subclass deficiency Autoimmunity Allergic asthma Specific antibodies Immunoglobulin therapy
Author Contribution Statement
Amrita Khokar retrieved and tabulated data from patient’s chart and electronic medical records. She wrote an initial draft of a small segment of text. Sudhir Gupta planned the study, supervised the collection of data, helped in the interpretation, and wrote majority of the manuscript.
This work was in part supported by Investigator-initiated Publication and Research Grant (PRG) from CSL Behring, King of Prussia, PA, and unrestricted funds from the Division of Basic and Clinical Immunology.
Compliance with Ethical Standards
No outside funding was obtained and is a retrospective study, and therefore, no consent from patients was required. Institutional Review Board (Human) approved this study.
Conflict of Interest
Both authors declare no conflict of interest.
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