Immunoglobulin G Subclass Deficiency is the Major Phenotype of Primary Immunodeficiency in a Korean Adult Cohort

Abstract

Primary immunodeficiency disease (PID) is a rare disorder in adults. Most often, serious forms are detected during infancy or childhood. However, mild forms of PID may not be diagnosed until later in life, and some types of humoral immunodeficiency may occur in adulthood. The purpose of this study was to identify clinical features of PID in Korean adults. A retrospective study was performed on 55 adult patients who were diagnosed as PID between January 1998 and January 2009 at a single tertiary medical center in Korea. IgG subclass deficiency was the most common phenotype (67%, 37/55), followed by total IgG deficiency (20%, 11/55), IgM deficiency (7%, 4/55), common variable immunodeficiency (2%, 1/55), and X-linked agammaglobulinemia (2%, 1/55). IgG3 and IgG4 were the most affected subclasses. Upper and lower respiratory tract infections (76%) were the most frequently observed symptoms, followed by multiple site infection (11%), urinary tract infection, and colitis. Bronchial asthma, rhinitis, and several autoimmune diseases were common associated diseases. IgG and IgG subclass deficiency should be considered in adult patients presenting with recurrent upper and lower respiratory infections, particularly in those with respiratory allergies or autoimmune diseases.

Association between primary immunodeficiency and asthma exacerbation in adult asthmatics

“…To the best of our knowledge, there have been few nationwide surveys of PID in an adult population in Korea, except one study reporting a PID prevalence of 1.13 per 100,000 in a pediatric population [1]. Although the reason the prevalence of PID is higher in adult asthmatics than in the general populations is not fully understood, it is conceivable that asthma is the most common comorbid condition in an adult PID cohort in Korea [4]; asthmatics with PID (especially IgG 3 SCD) suffered from recurrent respiratory infections, contributing to asthma exacerbation and asthma severity. Therefore, asthmatics with PID can be detected more frequently.…”

“…Furthermore, these repiratoty infections are known as major triggering factors for asthma exacerbation in adult asthmatics [3]. Recently, we demonstrated that immunoglobulin G 3 (IgG 3 ) subclass deficiency (IgG 3 SCD) is the most common type of PID associated with asthma exacerbation in adult asthmatics [4], in which monthly intravenous immunoglobulin (IVIG) therapy was beneficial for preventing asthma exacerbation [5]. We hypothesized that PID is associated with frequent asthma exacerbations and severe asthma in adult asthmatics.…”

Determination of immunological stress and oxygen free radical formation in white blood cells during allergen immunotherapy

“…IgG 3 subclass deficiency is closely associated with recurrent upper and lower respiratory infections, particularly in patients with respiratory allergy [18,19]. IgG3 is the predominant subclass involved in primary immune response to viral antigens, to M. Catarrhalis and S. pyogenes [20,21], as well as atypical pathogen infections and the exacerbations of asthma symptoms [19].…”

“…IgG3 is the predominant subclass involved in primary immune response to viral antigens, to M. Catarrhalis and S. pyogenes [20,21], as well as atypical pathogen infections and the exacerbations of asthma symptoms [19]. We did not find IgG3 deficiency, but the statistically significant increase after allergen immunotherapy could support several clinical findings that patients after such treatment better tolerate respiratory infections, not only due to suppression of allergic Th2 inflammation, but also due to changes in immune regulation (increased ROS in leukocytes, increased IgG3).…”

Immunoglobulin G4 deficiency can be a new entity for primary recurrent miscarriage: Successful pregnancy in two cases after treatment with intravenous immunoglobulin

“…Other disorders with IgG4 deficiency are Down syndrome, immune thrombocytopenia, lupus erythematosus, and growth hormone deficiency. Monthly intravenous immunoglobulins (IVIG) are the mainstay of treatment for symptomatic patients with IgG subclass deficiencies (9,10) . There are scant data on abnormal Ig subclasses in women with RM (11) .…”